Hair & Nails

The etiology of central centrifugal cicatricial alopecia (CCCA), a clinical and histological pattern of hair loss on the central scalp, has been well studied. This disease is chronic and progressive, with extensive follicular destruction and eventual burnout.^1,2 Central centrifugal cicatricial alopecia is most commonly seen in patients of African descent and has been shown to be 1 of the 5 most common dermatologic diagnoses in black patients.^3,4 The top 5 dermatologic diagnoses within this population include acne vulgaris (28.4%), dyschromia (19.9%), eczema (9.1%), alopecia (8.3%), and seborrheic dermatitis (6.7%).⁴ The incidence rate of CCCA is estimated to be 5.6%.^3,5 Most patients are women, with onset between the second and fourth decades of life.⁶

Central centrifugal cicatricial alopecia treatment efficacy is inversely correlated with disease duration. The primary goal of treatment is to prevent progression. Efforts are made to stimulate regrowth in areas that are not permanently scarred. When patients present with a substantial amount of scarring hair loss, dermatologists often are limited in their ability to achieve a cosmetically acceptable pattern of growth. Generally, hair is connected to a sense of self-worth in black women, and any type of hair loss has been shown to lead to frustration and decreased self-esteem.⁷ A 1994 study showed that 75% (44/58) of women with androgenetic alopecia had decreased self-esteem and 50% (29/58) had social challenges.⁸

The purpose of this pilot study was to determine the personal, historical, logistical, or environmental factors that preclude women from obtaining medical care for CCCA and to investigate how CCCA affects quality of life (QOL) and psychological well-being.

Methods

The investigators designed a survey study of adult, English-speaking, black women diagnosed with CCCA at the Northwestern University Department of Dermatology (Chicago, Illinois) between 2011 and 2017. Patients were selected from the electronic data warehouse compiled by the Department of Dermatology and were included if they fulfilled the following criteria: evaluated in the dermatology department between September 1, 2011, and September 30, 2017, by any faculty physician; diagnosed with CCCA; and aged 18 years or older. Patients were excluded if they did not speak English, as interpreters were not available. All patients who fulfilled the inclusion criteria provided signed informed consent prior to participation. All surveys were disseminated in the office or via telephone from fall 2016 to spring 2017 and took 10 to 15 minutes to complete. The research was approved by the authors’ institutional review board (IRB ID STU00203449).

Survey Instrument
The CCCA Barriers to Care and Quality of Life Survey is a 53-item survey instrument created by the study’s authors to measure 2 aspects of CCCA hair loss: barriers to medical care and QOL. The initial set of questions pertained to initial hair loss discovery and the number of physicians, both dermatologist and otherwise, seen for hair evaluation. Patients then rated their physician interactions on an ordinal Likert scale (1=poor, 2=fair, 3=good, 4=very good, 5=excellent). If patients saw more than 1 dermatologist or nondermatologist physician, ratings for each provider were included in our analysis. Patients listed the top 3 factors they considered when seeking medical care for their hair loss in order of importance. They also indicated how much they agreed with QOL statements on a Likert scale (1=strongly disagree, 2=disagree, 3=neutral, 4=agree, 5=strongly agree).

Data Analysis
Analyses were completed using data analysis software JMP Pro 13 from SAS and a Microsoft Excel spreadsheet. Continuous data were presented as mean, SD, median, minimum, and maximum. Categorical data were presented as counts and percentages. Nine QOL items were aggregated into a self-esteem category (questions 30–38).

Cronbach α, a statistical measure of internal consistency and how closely related items are in a group, was used to evaluate internal consistency reliability; values of 0.70 or greater indicate acceptable reliability.

Results

Of 501 individuals contacted, 34 completed the survey (7% completion rate). Nonrespondents included 7 who refused to participate and 460 who could not be contacted. All respondents self-identified as black women. Median age at time of survey administration was 46 years (range, 28–79 years); median age at CCCA diagnosis was 42 years (range, 15–73 years). Respondents did not significantly differ in age from nonrespondents (P=.46). The majority of respondents had an associate’s degree, bachelor’s degree, or advanced degree of education (master of arts, doctor of medicine, doctor of jurisprudence, doctor of philosophy); however, 8 women reported completing some college, 1 reported completing high school, and 1 reported no schooling. Three respondents had no health insurance.

Initial Hair Loss Discovery
The majority of respondents (22/34 [65%]) were first to notice their hair loss, while 5 (15%) reported hairstylists as the initial observers. Twelve respondents (35%) initially went to a physician to learn why they were losing hair; 6 (18%) instead utilized hairstylists or the Internet. Fifteen women (44%) waited more than 1 month up to 6 months after noticing hair loss before seeing a physician instead of going immediately within a 4-week period, and 16 (47%) waited 1 year or more.

Nondermatologist Consultation
Almost half (16/34 [47%]) of the women went to a nondermatologist physician regarding their hair loss; of them, half (8/16 [50%]) reported their physician did not examine the scalp, 3 (19%) reported their physician offered a biopsy, and none of them reported that their physician diagnosed them with CCCA. The median patient rating of their nondermatologist physician interactions was good (3 on a 5-point scale). Table 1 and Figure 1 show responses to individual items.

Dermatologist Consultation
All 34 respondents presented to a dermatologist. The majority of respondents (22/34 [65%]) saw either 1 or 2 dermatologists for their hair loss. Three (9%) reported their dermatologist did not examine their scalp. Twelve respondents (35%) reported their dermatologist did not offer a biopsy. Twenty-one respondents (62%) reported a CCCA diagnosis from the first dermatologist they saw. Twenty-three respondents (68%) were diagnosed by dermatologists with expertise in hair disorders. Sixteen (47%) were diagnosed by dermatologists within a skin-of-color center. Fourteen (41%) initial dermatology consultations were race concordant.

The median patient rating of their dermatologist interactions was excellent (5 on a 5-point scale). Table 2 and Figure 2 show responses to individual items. Respondents saw an average of 3 different providers, both dermatologists and otherwise.

Waiting to See a Dermatologist
Nearly all respondents (31/34 [91%]) recommended that other women with hair loss immediately go see a dermatologist.

Barriers to Care
The top 5 factors reported as most important when initially seeking care included the physician’s experience with black hair and CCCA, the patient’s personal hairstyling practices, the physician’s ethnicity, availability of effective treatment options, and treatment cost. Table 3 shows frequency counts for these freely reported factors.

Quality of Life
The median score on 9 aggregated self-esteem items was 4 on a 5-point scale, representing an agree response to statements such as “I feel embarrassed, self-conscious, or frustrated about my hair loss” (28/34 [82%]) and “My hair loss bothers me” (28/34 [82%])(Table 4). Cronbach α for self-esteem survey items was 0.7826.

For the nonaggregated items, many respondents strongly disagreed with statements pertaining to activities of daily living, including “I take care of where I sit or stand at social gatherings due to my hair loss” (18/34 [53%]), “My hair loss makes it difficult for me to go to the grocery store” (29/34 [85%]), “My hair loss makes it difficult for me to attend faith-based activities” (30/34 [88%]), “My hair loss makes it difficult for me to exercise” (23/34 [68%]), “My hair loss makes it difficult for me to go to work and/or school” (24/34 [71%]), “My hair loss makes it difficult for me to go out with a significant other” (24/34 [71%]), “My hair loss makes it difficult for me to spend time with family” (27/34 [79%]), and “My hair loss makes it difficult for me to go to a hairstylist” (16/34 [47%]).

Comment

The majority of respondents were first to discover their hair loss. Harbingers of CCCA hair loss include paresthesia, tenderness, and itch,⁶ symptoms that are hard to ignore. Unfortunately, many patients notice hair thinning years after the scarring process has begun and a notable amount of hair has already been lost.^6,9

Fifteen percent of respondents learned about their hair loss from their hairstylist. Women of African descent often maintain hairstyles that require frequent interactions with a hair care professional.^7,10 As a result, hairstylists are at the forefront of early alopecia detection and are a valued resource in the black community. Open dialogue between dermatologists and hair care professionals could funnel women with hair loss into treatment before extensive damage.

Fifteen women (44%) recalled a waiting period of several months before seeking medical assistance, and 16 (47%) reported waiting 1 year or more. However, 91% of respondents indicated that women with hair loss should immediately see a physician for evaluation, thus patient experiences underscore the importance of early treatment. In our experience, many patients wait years before presenting to a physician. Some work with their hairstylists first to address the issue, while others do not realize how notable the loss has become. Some have a negative experience with one provider or are told there is nothing that can be done and then wait many years to see a second provider. Proper education of patients, physicians, and hairstylists is important in the identification and prompt treatment of this condition.

It is perhaps to be expected that patients rated interactions with dermatologists as excellent and very good more frequently than interactions with nondermatologists, which may be due to an absence of thorough hair evaluation with nondermatologists. Respondents reported that only half of nondermatologist providers actually examined their scalp during an initial encounter. However, both physician groups had the lowest frequencies of excellent and very good ratings on “understanding of your hair” (Tables 1 and 2). Patients with hair loss seek immediate answers, and often it is the specialist that can give them a firm diagnosis as opposed to a primary care provider. The fact that dermatologists and nondermatologists alike scored poorly on patient-perceived understanding of CCCA indicates an area for improvement within patient-physician interactions and physician knowledge.

The top 5 factors important to respondents when obtaining medical care included the physician’s experience with black hair and CCCA, the patient’s personal hairstyling practices, the physician’s ethnicity, availability of effective treatment options, and treatment cost. Patients with CCCA seeing dermatologists may discern a lack of experience with ethnic hair that leads patients to doubt their physicians’ ability to provide adequate care and decreased shared decision-making.^11,12 These patient perceptions are not unfounded; a 2008 study showed that dermatology residents are not uniformly trained in diseases pertaining to patients with skin of color.¹³ Thus, incorporation of education on skin of color in dermatology training programs is critical.

Finally, hair loss patients often have concerns regarding how medical therapeutics could adversely affect personal hair care regimens, including washing and hairstyling practices. Current research demonstrates that patients consider treatment effectiveness and ability to be integrated into daily routines after establishing medical care.¹⁴ The present study shows that some CCCA patients contemplate how well a therapy will work before seeking medical care, demonstrating that patients continue to have these concerns after establishing medical care. Consideration of treatment effectiveness is important for both patients and providers, as there is minimal evidence behind current CCCA management practices. The ability for treatments to be easily integrated into daily hair care habits is important to maintain patient compliance.

Participants’ median self-esteem scores indicate the effect of CCCA on morale and self-perception. Items scrutinizing this construct had acceptable internal consistency reliability. It is interesting to note that activities of daily living were not impacted by hair loss. Examination of self-esteem is important in the alopecia population because the effect of hair loss on mental status is well documented.^15-17 Low self-esteem has been reported as a prospective risk factor for clinical depression.^18-20 In black patients, clinical depression rates surpass those of Hispanics and non-Hispanic white individuals.²¹ Dermatologists must consider the psychological status of all patients, particularly populations at risk for severe disease.

Limitations of this study include the small (34 participants) and mostly highly educated sample size, limited survey validity, and potential patient bias. Because many patients changed their address and/or telephone number in the time between CCCA diagnosis and the present study, we were left with a small pilot study, which minimizes the impact of our findings. Furthermore, our survey was created by a single expert’s opinion and modeling from preexisting alopecia questionnaires¹⁶; full validity procedures analyzing face, content, and criterion validity were not undertaken. Finally, the majority of respondents were patients of one of the study’s authors (S.S.L.P.), which could influence survey responses. The fact that some providers were hair experts and some were race concordant with their patients also could potentially affect the responses received, which was not analyzed in the present study. Future studies with more respondents from multiple providers would help clarify our preliminary findings.

Conclusion

Analysis of barriers to care and QOL in patients with skin of color is an essential addition to dermatologic discourse. Alopecia is particularly important to investigate, as prior research has found it to be one of the top 5 diagnoses made in patients with skin of color.^3,4 Alopecia has been shown to negatively affect QOL.^15,22,23 This study, although limited by small sample size, suggests CCCA also is a contributor to self-esteem challenges, similar to other forms of hair loss. Patient-physician interactions and personal hairstyling practices are prominent barriers to care for CCCA patients, demonstrating the need for quality education on skin of color and cultural competency in dermatology residencies across the country.

The etiology of central centrifugal cicatricial alopecia (CCCA), a clinical and histological pattern of hair loss on the central scalp, has been well studied. This disease is chronic and progressive, with extensive follicular destruction and eventual burnout.^1,2 Central centrifugal cicatricial alopecia is most commonly seen in patients of African descent and has been shown to be 1 of the 5 most common dermatologic diagnoses in black patients.^3,4 The top 5 dermatologic diagnoses within this population include acne vulgaris (28.4%), dyschromia (19.9%), eczema (9.1%), alopecia (8.3%), and seborrheic dermatitis (6.7%).⁴ The incidence rate of CCCA is estimated to be 5.6%.^3,5 Most patients are women, with onset between the second and fourth decades of life.⁶

Central centrifugal cicatricial alopecia treatment efficacy is inversely correlated with disease duration. The primary goal of treatment is to prevent progression. Efforts are made to stimulate regrowth in areas that are not permanently scarred. When patients present with a substantial amount of scarring hair loss, dermatologists often are limited in their ability to achieve a cosmetically acceptable pattern of growth. Generally, hair is connected to a sense of self-worth in black women, and any type of hair loss has been shown to lead to frustration and decreased self-esteem.⁷ A 1994 study showed that 75% (44/58) of women with androgenetic alopecia had decreased self-esteem and 50% (29/58) had social challenges.⁸

The purpose of this pilot study was to determine the personal, historical, logistical, or environmental factors that preclude women from obtaining medical care for CCCA and to investigate how CCCA affects quality of life (QOL) and psychological well-being.

Methods

The investigators designed a survey study of adult, English-speaking, black women diagnosed with CCCA at the Northwestern University Department of Dermatology (Chicago, Illinois) between 2011 and 2017. Patients were selected from the electronic data warehouse compiled by the Department of Dermatology and were included if they fulfilled the following criteria: evaluated in the dermatology department between September 1, 2011, and September 30, 2017, by any faculty physician; diagnosed with CCCA; and aged 18 years or older. Patients were excluded if they did not speak English, as interpreters were not available. All patients who fulfilled the inclusion criteria provided signed informed consent prior to participation. All surveys were disseminated in the office or via telephone from fall 2016 to spring 2017 and took 10 to 15 minutes to complete. The research was approved by the authors’ institutional review board (IRB ID STU00203449).

Survey Instrument
The CCCA Barriers to Care and Quality of Life Survey is a 53-item survey instrument created by the study’s authors to measure 2 aspects of CCCA hair loss: barriers to medical care and QOL. The initial set of questions pertained to initial hair loss discovery and the number of physicians, both dermatologist and otherwise, seen for hair evaluation. Patients then rated their physician interactions on an ordinal Likert scale (1=poor, 2=fair, 3=good, 4=very good, 5=excellent). If patients saw more than 1 dermatologist or nondermatologist physician, ratings for each provider were included in our analysis. Patients listed the top 3 factors they considered when seeking medical care for their hair loss in order of importance. They also indicated how much they agreed with QOL statements on a Likert scale (1=strongly disagree, 2=disagree, 3=neutral, 4=agree, 5=strongly agree).

Data Analysis
Analyses were completed using data analysis software JMP Pro 13 from SAS and a Microsoft Excel spreadsheet. Continuous data were presented as mean, SD, median, minimum, and maximum. Categorical data were presented as counts and percentages. Nine QOL items were aggregated into a self-esteem category (questions 30–38).

Cronbach α, a statistical measure of internal consistency and how closely related items are in a group, was used to evaluate internal consistency reliability; values of 0.70 or greater indicate acceptable reliability.

Results

Of 501 individuals contacted, 34 completed the survey (7% completion rate). Nonrespondents included 7 who refused to participate and 460 who could not be contacted. All respondents self-identified as black women. Median age at time of survey administration was 46 years (range, 28–79 years); median age at CCCA diagnosis was 42 years (range, 15–73 years). Respondents did not significantly differ in age from nonrespondents (P=.46). The majority of respondents had an associate’s degree, bachelor’s degree, or advanced degree of education (master of arts, doctor of medicine, doctor of jurisprudence, doctor of philosophy); however, 8 women reported completing some college, 1 reported completing high school, and 1 reported no schooling. Three respondents had no health insurance.

Initial Hair Loss Discovery
The majority of respondents (22/34 [65%]) were first to notice their hair loss, while 5 (15%) reported hairstylists as the initial observers. Twelve respondents (35%) initially went to a physician to learn why they were losing hair; 6 (18%) instead utilized hairstylists or the Internet. Fifteen women (44%) waited more than 1 month up to 6 months after noticing hair loss before seeing a physician instead of going immediately within a 4-week period, and 16 (47%) waited 1 year or more.

Nondermatologist Consultation
Almost half (16/34 [47%]) of the women went to a nondermatologist physician regarding their hair loss; of them, half (8/16 [50%]) reported their physician did not examine the scalp, 3 (19%) reported their physician offered a biopsy, and none of them reported that their physician diagnosed them with CCCA. The median patient rating of their nondermatologist physician interactions was good (3 on a 5-point scale). Table 1 and Figure 1 show responses to individual items.

Dermatologist Consultation
All 34 respondents presented to a dermatologist. The majority of respondents (22/34 [65%]) saw either 1 or 2 dermatologists for their hair loss. Three (9%) reported their dermatologist did not examine their scalp. Twelve respondents (35%) reported their dermatologist did not offer a biopsy. Twenty-one respondents (62%) reported a CCCA diagnosis from the first dermatologist they saw. Twenty-three respondents (68%) were diagnosed by dermatologists with expertise in hair disorders. Sixteen (47%) were diagnosed by dermatologists within a skin-of-color center. Fourteen (41%) initial dermatology consultations were race concordant.

The median patient rating of their dermatologist interactions was excellent (5 on a 5-point scale). Table 2 and Figure 2 show responses to individual items. Respondents saw an average of 3 different providers, both dermatologists and otherwise.

Waiting to See a Dermatologist
Nearly all respondents (31/34 [91%]) recommended that other women with hair loss immediately go see a dermatologist.

Barriers to Care
The top 5 factors reported as most important when initially seeking care included the physician’s experience with black hair and CCCA, the patient’s personal hairstyling practices, the physician’s ethnicity, availability of effective treatment options, and treatment cost. Table 3 shows frequency counts for these freely reported factors.

Quality of Life
The median score on 9 aggregated self-esteem items was 4 on a 5-point scale, representing an agree response to statements such as “I feel embarrassed, self-conscious, or frustrated about my hair loss” (28/34 [82%]) and “My hair loss bothers me” (28/34 [82%])(Table 4). Cronbach α for self-esteem survey items was 0.7826.

For the nonaggregated items, many respondents strongly disagreed with statements pertaining to activities of daily living, including “I take care of where I sit or stand at social gatherings due to my hair loss” (18/34 [53%]), “My hair loss makes it difficult for me to go to the grocery store” (29/34 [85%]), “My hair loss makes it difficult for me to attend faith-based activities” (30/34 [88%]), “My hair loss makes it difficult for me to exercise” (23/34 [68%]), “My hair loss makes it difficult for me to go to work and/or school” (24/34 [71%]), “My hair loss makes it difficult for me to go out with a significant other” (24/34 [71%]), “My hair loss makes it difficult for me to spend time with family” (27/34 [79%]), and “My hair loss makes it difficult for me to go to a hairstylist” (16/34 [47%]).

Comment

The majority of respondents were first to discover their hair loss. Harbingers of CCCA hair loss include paresthesia, tenderness, and itch,⁶ symptoms that are hard to ignore. Unfortunately, many patients notice hair thinning years after the scarring process has begun and a notable amount of hair has already been lost.^6,9

Fifteen percent of respondents learned about their hair loss from their hairstylist. Women of African descent often maintain hairstyles that require frequent interactions with a hair care professional.^7,10 As a result, hairstylists are at the forefront of early alopecia detection and are a valued resource in the black community. Open dialogue between dermatologists and hair care professionals could funnel women with hair loss into treatment before extensive damage.

Fifteen women (44%) recalled a waiting period of several months before seeking medical assistance, and 16 (47%) reported waiting 1 year or more. However, 91% of respondents indicated that women with hair loss should immediately see a physician for evaluation, thus patient experiences underscore the importance of early treatment. In our experience, many patients wait years before presenting to a physician. Some work with their hairstylists first to address the issue, while others do not realize how notable the loss has become. Some have a negative experience with one provider or are told there is nothing that can be done and then wait many years to see a second provider. Proper education of patients, physicians, and hairstylists is important in the identification and prompt treatment of this condition.

It is perhaps to be expected that patients rated interactions with dermatologists as excellent and very good more frequently than interactions with nondermatologists, which may be due to an absence of thorough hair evaluation with nondermatologists. Respondents reported that only half of nondermatologist providers actually examined their scalp during an initial encounter. However, both physician groups had the lowest frequencies of excellent and very good ratings on “understanding of your hair” (Tables 1 and 2). Patients with hair loss seek immediate answers, and often it is the specialist that can give them a firm diagnosis as opposed to a primary care provider. The fact that dermatologists and nondermatologists alike scored poorly on patient-perceived understanding of CCCA indicates an area for improvement within patient-physician interactions and physician knowledge.

The top 5 factors important to respondents when obtaining medical care included the physician’s experience with black hair and CCCA, the patient’s personal hairstyling practices, the physician’s ethnicity, availability of effective treatment options, and treatment cost. Patients with CCCA seeing dermatologists may discern a lack of experience with ethnic hair that leads patients to doubt their physicians’ ability to provide adequate care and decreased shared decision-making.^11,12 These patient perceptions are not unfounded; a 2008 study showed that dermatology residents are not uniformly trained in diseases pertaining to patients with skin of color.¹³ Thus, incorporation of education on skin of color in dermatology training programs is critical.

Finally, hair loss patients often have concerns regarding how medical therapeutics could adversely affect personal hair care regimens, including washing and hairstyling practices. Current research demonstrates that patients consider treatment effectiveness and ability to be integrated into daily routines after establishing medical care.¹⁴ The present study shows that some CCCA patients contemplate how well a therapy will work before seeking medical care, demonstrating that patients continue to have these concerns after establishing medical care. Consideration of treatment effectiveness is important for both patients and providers, as there is minimal evidence behind current CCCA management practices. The ability for treatments to be easily integrated into daily hair care habits is important to maintain patient compliance.

Participants’ median self-esteem scores indicate the effect of CCCA on morale and self-perception. Items scrutinizing this construct had acceptable internal consistency reliability. It is interesting to note that activities of daily living were not impacted by hair loss. Examination of self-esteem is important in the alopecia population because the effect of hair loss on mental status is well documented.^15-17 Low self-esteem has been reported as a prospective risk factor for clinical depression.^18-20 In black patients, clinical depression rates surpass those of Hispanics and non-Hispanic white individuals.²¹ Dermatologists must consider the psychological status of all patients, particularly populations at risk for severe disease.

Limitations of this study include the small (34 participants) and mostly highly educated sample size, limited survey validity, and potential patient bias. Because many patients changed their address and/or telephone number in the time between CCCA diagnosis and the present study, we were left with a small pilot study, which minimizes the impact of our findings. Furthermore, our survey was created by a single expert’s opinion and modeling from preexisting alopecia questionnaires¹⁶; full validity procedures analyzing face, content, and criterion validity were not undertaken. Finally, the majority of respondents were patients of one of the study’s authors (S.S.L.P.), which could influence survey responses. The fact that some providers were hair experts and some were race concordant with their patients also could potentially affect the responses received, which was not analyzed in the present study. Future studies with more respondents from multiple providers would help clarify our preliminary findings.

Conclusion

Analysis of barriers to care and QOL in patients with skin of color is an essential addition to dermatologic discourse. Alopecia is particularly important to investigate, as prior research has found it to be one of the top 5 diagnoses made in patients with skin of color.^3,4 Alopecia has been shown to negatively affect QOL.^15,22,23 This study, although limited by small sample size, suggests CCCA also is a contributor to self-esteem challenges, similar to other forms of hair loss. Patient-physician interactions and personal hairstyling practices are prominent barriers to care for CCCA patients, demonstrating the need for quality education on skin of color and cultural competency in dermatology residencies across the country.

The etiology of central centrifugal cicatricial alopecia (CCCA), a clinical and histological pattern of hair loss on the central scalp, has been well studied. This disease is chronic and progressive, with extensive follicular destruction and eventual burnout.^1,2 Central centrifugal cicatricial alopecia is most commonly seen in patients of African descent and has been shown to be 1 of the 5 most common dermatologic diagnoses in black patients.^3,4 The top 5 dermatologic diagnoses within this population include acne vulgaris (28.4%), dyschromia (19.9%), eczema (9.1%), alopecia (8.3%), and seborrheic dermatitis (6.7%).⁴ The incidence rate of CCCA is estimated to be 5.6%.^3,5 Most patients are women, with onset between the second and fourth decades of life.⁶

Central centrifugal cicatricial alopecia treatment efficacy is inversely correlated with disease duration. The primary goal of treatment is to prevent progression. Efforts are made to stimulate regrowth in areas that are not permanently scarred. When patients present with a substantial amount of scarring hair loss, dermatologists often are limited in their ability to achieve a cosmetically acceptable pattern of growth. Generally, hair is connected to a sense of self-worth in black women, and any type of hair loss has been shown to lead to frustration and decreased self-esteem.⁷ A 1994 study showed that 75% (44/58) of women with androgenetic alopecia had decreased self-esteem and 50% (29/58) had social challenges.⁸

The purpose of this pilot study was to determine the personal, historical, logistical, or environmental factors that preclude women from obtaining medical care for CCCA and to investigate how CCCA affects quality of life (QOL) and psychological well-being.

Methods

The investigators designed a survey study of adult, English-speaking, black women diagnosed with CCCA at the Northwestern University Department of Dermatology (Chicago, Illinois) between 2011 and 2017. Patients were selected from the electronic data warehouse compiled by the Department of Dermatology and were included if they fulfilled the following criteria: evaluated in the dermatology department between September 1, 2011, and September 30, 2017, by any faculty physician; diagnosed with CCCA; and aged 18 years or older. Patients were excluded if they did not speak English, as interpreters were not available. All patients who fulfilled the inclusion criteria provided signed informed consent prior to participation. All surveys were disseminated in the office or via telephone from fall 2016 to spring 2017 and took 10 to 15 minutes to complete. The research was approved by the authors’ institutional review board (IRB ID STU00203449).

Survey Instrument
The CCCA Barriers to Care and Quality of Life Survey is a 53-item survey instrument created by the study’s authors to measure 2 aspects of CCCA hair loss: barriers to medical care and QOL. The initial set of questions pertained to initial hair loss discovery and the number of physicians, both dermatologist and otherwise, seen for hair evaluation. Patients then rated their physician interactions on an ordinal Likert scale (1=poor, 2=fair, 3=good, 4=very good, 5=excellent). If patients saw more than 1 dermatologist or nondermatologist physician, ratings for each provider were included in our analysis. Patients listed the top 3 factors they considered when seeking medical care for their hair loss in order of importance. They also indicated how much they agreed with QOL statements on a Likert scale (1=strongly disagree, 2=disagree, 3=neutral, 4=agree, 5=strongly agree).

Data Analysis
Analyses were completed using data analysis software JMP Pro 13 from SAS and a Microsoft Excel spreadsheet. Continuous data were presented as mean, SD, median, minimum, and maximum. Categorical data were presented as counts and percentages. Nine QOL items were aggregated into a self-esteem category (questions 30–38).

Cronbach α, a statistical measure of internal consistency and how closely related items are in a group, was used to evaluate internal consistency reliability; values of 0.70 or greater indicate acceptable reliability.

Results

Of 501 individuals contacted, 34 completed the survey (7% completion rate). Nonrespondents included 7 who refused to participate and 460 who could not be contacted. All respondents self-identified as black women. Median age at time of survey administration was 46 years (range, 28–79 years); median age at CCCA diagnosis was 42 years (range, 15–73 years). Respondents did not significantly differ in age from nonrespondents (P=.46). The majority of respondents had an associate’s degree, bachelor’s degree, or advanced degree of education (master of arts, doctor of medicine, doctor of jurisprudence, doctor of philosophy); however, 8 women reported completing some college, 1 reported completing high school, and 1 reported no schooling. Three respondents had no health insurance.

Initial Hair Loss Discovery
The majority of respondents (22/34 [65%]) were first to notice their hair loss, while 5 (15%) reported hairstylists as the initial observers. Twelve respondents (35%) initially went to a physician to learn why they were losing hair; 6 (18%) instead utilized hairstylists or the Internet. Fifteen women (44%) waited more than 1 month up to 6 months after noticing hair loss before seeing a physician instead of going immediately within a 4-week period, and 16 (47%) waited 1 year or more.

Nondermatologist Consultation
Almost half (16/34 [47%]) of the women went to a nondermatologist physician regarding their hair loss; of them, half (8/16 [50%]) reported their physician did not examine the scalp, 3 (19%) reported their physician offered a biopsy, and none of them reported that their physician diagnosed them with CCCA. The median patient rating of their nondermatologist physician interactions was good (3 on a 5-point scale). Table 1 and Figure 1 show responses to individual items.

Dermatologist Consultation
All 34 respondents presented to a dermatologist. The majority of respondents (22/34 [65%]) saw either 1 or 2 dermatologists for their hair loss. Three (9%) reported their dermatologist did not examine their scalp. Twelve respondents (35%) reported their dermatologist did not offer a biopsy. Twenty-one respondents (62%) reported a CCCA diagnosis from the first dermatologist they saw. Twenty-three respondents (68%) were diagnosed by dermatologists with expertise in hair disorders. Sixteen (47%) were diagnosed by dermatologists within a skin-of-color center. Fourteen (41%) initial dermatology consultations were race concordant.

The median patient rating of their dermatologist interactions was excellent (5 on a 5-point scale). Table 2 and Figure 2 show responses to individual items. Respondents saw an average of 3 different providers, both dermatologists and otherwise.

Waiting to See a Dermatologist
Nearly all respondents (31/34 [91%]) recommended that other women with hair loss immediately go see a dermatologist.

Barriers to Care
The top 5 factors reported as most important when initially seeking care included the physician’s experience with black hair and CCCA, the patient’s personal hairstyling practices, the physician’s ethnicity, availability of effective treatment options, and treatment cost. Table 3 shows frequency counts for these freely reported factors.

Quality of Life
The median score on 9 aggregated self-esteem items was 4 on a 5-point scale, representing an agree response to statements such as “I feel embarrassed, self-conscious, or frustrated about my hair loss” (28/34 [82%]) and “My hair loss bothers me” (28/34 [82%])(Table 4). Cronbach α for self-esteem survey items was 0.7826.

For the nonaggregated items, many respondents strongly disagreed with statements pertaining to activities of daily living, including “I take care of where I sit or stand at social gatherings due to my hair loss” (18/34 [53%]), “My hair loss makes it difficult for me to go to the grocery store” (29/34 [85%]), “My hair loss makes it difficult for me to attend faith-based activities” (30/34 [88%]), “My hair loss makes it difficult for me to exercise” (23/34 [68%]), “My hair loss makes it difficult for me to go to work and/or school” (24/34 [71%]), “My hair loss makes it difficult for me to go out with a significant other” (24/34 [71%]), “My hair loss makes it difficult for me to spend time with family” (27/34 [79%]), and “My hair loss makes it difficult for me to go to a hairstylist” (16/34 [47%]).

Comment

The majority of respondents were first to discover their hair loss. Harbingers of CCCA hair loss include paresthesia, tenderness, and itch,⁶ symptoms that are hard to ignore. Unfortunately, many patients notice hair thinning years after the scarring process has begun and a notable amount of hair has already been lost.^6,9

Fifteen percent of respondents learned about their hair loss from their hairstylist. Women of African descent often maintain hairstyles that require frequent interactions with a hair care professional.^7,10 As a result, hairstylists are at the forefront of early alopecia detection and are a valued resource in the black community. Open dialogue between dermatologists and hair care professionals could funnel women with hair loss into treatment before extensive damage.

Fifteen women (44%) recalled a waiting period of several months before seeking medical assistance, and 16 (47%) reported waiting 1 year or more. However, 91% of respondents indicated that women with hair loss should immediately see a physician for evaluation, thus patient experiences underscore the importance of early treatment. In our experience, many patients wait years before presenting to a physician. Some work with their hairstylists first to address the issue, while others do not realize how notable the loss has become. Some have a negative experience with one provider or are told there is nothing that can be done and then wait many years to see a second provider. Proper education of patients, physicians, and hairstylists is important in the identification and prompt treatment of this condition.

It is perhaps to be expected that patients rated interactions with dermatologists as excellent and very good more frequently than interactions with nondermatologists, which may be due to an absence of thorough hair evaluation with nondermatologists. Respondents reported that only half of nondermatologist providers actually examined their scalp during an initial encounter. However, both physician groups had the lowest frequencies of excellent and very good ratings on “understanding of your hair” (Tables 1 and 2). Patients with hair loss seek immediate answers, and often it is the specialist that can give them a firm diagnosis as opposed to a primary care provider. The fact that dermatologists and nondermatologists alike scored poorly on patient-perceived understanding of CCCA indicates an area for improvement within patient-physician interactions and physician knowledge.

The top 5 factors important to respondents when obtaining medical care included the physician’s experience with black hair and CCCA, the patient’s personal hairstyling practices, the physician’s ethnicity, availability of effective treatment options, and treatment cost. Patients with CCCA seeing dermatologists may discern a lack of experience with ethnic hair that leads patients to doubt their physicians’ ability to provide adequate care and decreased shared decision-making.^11,12 These patient perceptions are not unfounded; a 2008 study showed that dermatology residents are not uniformly trained in diseases pertaining to patients with skin of color.¹³ Thus, incorporation of education on skin of color in dermatology training programs is critical.

Finally, hair loss patients often have concerns regarding how medical therapeutics could adversely affect personal hair care regimens, including washing and hairstyling practices. Current research demonstrates that patients consider treatment effectiveness and ability to be integrated into daily routines after establishing medical care.¹⁴ The present study shows that some CCCA patients contemplate how well a therapy will work before seeking medical care, demonstrating that patients continue to have these concerns after establishing medical care. Consideration of treatment effectiveness is important for both patients and providers, as there is minimal evidence behind current CCCA management practices. The ability for treatments to be easily integrated into daily hair care habits is important to maintain patient compliance.

Participants’ median self-esteem scores indicate the effect of CCCA on morale and self-perception. Items scrutinizing this construct had acceptable internal consistency reliability. It is interesting to note that activities of daily living were not impacted by hair loss. Examination of self-esteem is important in the alopecia population because the effect of hair loss on mental status is well documented.^15-17 Low self-esteem has been reported as a prospective risk factor for clinical depression.^18-20 In black patients, clinical depression rates surpass those of Hispanics and non-Hispanic white individuals.²¹ Dermatologists must consider the psychological status of all patients, particularly populations at risk for severe disease.

Limitations of this study include the small (34 participants) and mostly highly educated sample size, limited survey validity, and potential patient bias. Because many patients changed their address and/or telephone number in the time between CCCA diagnosis and the present study, we were left with a small pilot study, which minimizes the impact of our findings. Furthermore, our survey was created by a single expert’s opinion and modeling from preexisting alopecia questionnaires¹⁶; full validity procedures analyzing face, content, and criterion validity were not undertaken. Finally, the majority of respondents were patients of one of the study’s authors (S.S.L.P.), which could influence survey responses. The fact that some providers were hair experts and some were race concordant with their patients also could potentially affect the responses received, which was not analyzed in the present study. Future studies with more respondents from multiple providers would help clarify our preliminary findings.

Conclusion

Analysis of barriers to care and QOL in patients with skin of color is an essential addition to dermatologic discourse. Alopecia is particularly important to investigate, as prior research has found it to be one of the top 5 diagnoses made in patients with skin of color.^3,4 Alopecia has been shown to negatively affect QOL.^15,22,23 This study, although limited by small sample size, suggests CCCA also is a contributor to self-esteem challenges, similar to other forms of hair loss. Patient-physician interactions and personal hairstyling practices are prominent barriers to care for CCCA patients, demonstrating the need for quality education on skin of color and cultural competency in dermatology residencies across the country.

LAS VEGAS – Alopecia is hard to bear for patients and has been difficult to treat, but “there’s been a revolution in thinking about how to treat alopecia areata,” according to Maria Hordinsky, MD.

Dr. Hordinsky, professor and chair of the department of dermatology, University of Minnesota, Minneapolis, discussed hair disorders in multiple presentations at Skin Disease Education Foundation’s annual Las Vegas Dermatology Seminar. In an interview after her session on alopecia areata, she elaborated on the state of research and treatment for patients with this diagnosis.



DERMATOLOGY NEWS: What has changed in alopecia areata treatment over the last few years?

Dr. Hordinsky: There is still no Food and Drug Administration–approved treatment for this disease. But recent studies have helped us understand how alopecia areata occurs, how important interleukin-15 is, and how to target this cytokine as well as others. At the same time, expertise has developed at several centers across the United States with using the Janus kinase inhibitor tofacitinib [Xeljanz] at 5 milligrams twice a day. This has led to more off-label use. Concurrently, many pharmaceutical companies with interest in alopecia areata have begun to study different JAK inhibitors that target different cytokine receptors. Researchers also are exploring topical JAK inhibitors.



DN: What about the high costs of these drugs?

Dr. Hordinsky: Some insurers are covering tofacitinib, and patient assistance programs are very helpful and beneficial. In my own practice, most patients taking these drugs are using those programs.



DN: Are any treatments being used less than in the past?

Dr. Hordinsky: We’re still using tools that we have in the toolbox because we don’t have an approved treatment. We use topical steroids and intralesional steroids. We also use prednisone as needed, and we use contact sensitization therapy.



DN: In your presentation, you talked about alopecia areata in body areas outside of the scalp. What should dermatologists know about the eyebrows in patients with alopecia areata?

Dr. Hordinsky: Some patients don’t care as much about the scalp hair loss because they’ve figured out how to deal with it. What really bothers some patients is their eyebrow hair loss. You can think of situations where alopecia areata creates a circle in the middle of the eyebrow on the left side, but not the right, or you lose one eyebrow but not the other. We use techniques such as intralesional steroids. If there’s some hair growth present that’s lightly pigmented, we may apply topical minoxidil or Latisse [bimatoprost] to the brow area. Patients may also do microblading.



DN: Are there eyebrow prosthetics?

Dr. Hordinsky: Yes, there are. The National Alopecia Areata Foundation provides a lot of information to patients and providers about these devices. There are devices that you can tape on your brow area. Some don’t look great cosmetically, but some look fantastic. Microblading may create the most normal appearance.



DN: What about eyelashes?

Dr. Hordinsky: Eyelash loss is tough and really bothersome to patients if they don’t wear glasses because of the protection provided by eyelashes, such as when you blink against airborne dust. If there is some hair present in the eyebrow regions, one can try to regrow hair and use something that’s safe in that region, like topical Latisse. These treatments have to be tried for a couple of months before you say yea or nay, and you and the patient have to have reasonable expectations.



DN: What about men’s beards?

Dr. Hordinsky: You can treat those areas with topical or intralesional steroids. My own experience is that you have to use intralesional steroids, and overall, this form of alopecia areata may be one of the most difficult to manage successfully.



DN: Do men complain about missing chest hair?

Dr. Hordinsky: Chest hair doesn’t come up a lot for me. For men, it’s mainly the beard area. But people with alopecia areata may sometimes minimize or not bring up discussion of loss of hair in the underarms, the genital region, or the chest. It may be because they’ve figured out how to deal with it.



DN: How do you think treatments will improve over the next 5-10 years?

Dr. Hordinsky: We have a number of companies putting JAK inhibitors into clinical trials. A major step forward will be figuring out which one works the best, and then the next hurdle will be sustainability. There are very few studies in alopecia areata about how long a response to treatment can be maintained. Another big step will be the development of a topical agent that is able to penetrate through the skin to the level of the immune attack at the lower part of the hair follicle and provide the opportunity to possibly not only grow hair but also to maintain hair growth. So there’s a lot of evolution going on right now.

Dr. Hordinsky disclosed consulting work with Procter & Gamble, Concert, and Cassiopea, and grant/research support from Aclaris, National Alopecia Areata Foundation, Allergan.

SDEF and this news organization are owned by the same parent company.

LAS VEGAS – Alopecia is hard to bear for patients and has been difficult to treat, but “there’s been a revolution in thinking about how to treat alopecia areata,” according to Maria Hordinsky, MD.

Dr. Hordinsky, professor and chair of the department of dermatology, University of Minnesota, Minneapolis, discussed hair disorders in multiple presentations at Skin Disease Education Foundation’s annual Las Vegas Dermatology Seminar. In an interview after her session on alopecia areata, she elaborated on the state of research and treatment for patients with this diagnosis.



DERMATOLOGY NEWS: What has changed in alopecia areata treatment over the last few years?

Dr. Hordinsky: There is still no Food and Drug Administration–approved treatment for this disease. But recent studies have helped us understand how alopecia areata occurs, how important interleukin-15 is, and how to target this cytokine as well as others. At the same time, expertise has developed at several centers across the United States with using the Janus kinase inhibitor tofacitinib [Xeljanz] at 5 milligrams twice a day. This has led to more off-label use. Concurrently, many pharmaceutical companies with interest in alopecia areata have begun to study different JAK inhibitors that target different cytokine receptors. Researchers also are exploring topical JAK inhibitors.



DN: What about the high costs of these drugs?

Dr. Hordinsky: Some insurers are covering tofacitinib, and patient assistance programs are very helpful and beneficial. In my own practice, most patients taking these drugs are using those programs.



DN: Are any treatments being used less than in the past?

Dr. Hordinsky: We’re still using tools that we have in the toolbox because we don’t have an approved treatment. We use topical steroids and intralesional steroids. We also use prednisone as needed, and we use contact sensitization therapy.



DN: In your presentation, you talked about alopecia areata in body areas outside of the scalp. What should dermatologists know about the eyebrows in patients with alopecia areata?

Dr. Hordinsky: Some patients don’t care as much about the scalp hair loss because they’ve figured out how to deal with it. What really bothers some patients is their eyebrow hair loss. You can think of situations where alopecia areata creates a circle in the middle of the eyebrow on the left side, but not the right, or you lose one eyebrow but not the other. We use techniques such as intralesional steroids. If there’s some hair growth present that’s lightly pigmented, we may apply topical minoxidil or Latisse [bimatoprost] to the brow area. Patients may also do microblading.



DN: Are there eyebrow prosthetics?

Dr. Hordinsky: Yes, there are. The National Alopecia Areata Foundation provides a lot of information to patients and providers about these devices. There are devices that you can tape on your brow area. Some don’t look great cosmetically, but some look fantastic. Microblading may create the most normal appearance.



DN: What about eyelashes?

Dr. Hordinsky: Eyelash loss is tough and really bothersome to patients if they don’t wear glasses because of the protection provided by eyelashes, such as when you blink against airborne dust. If there is some hair present in the eyebrow regions, one can try to regrow hair and use something that’s safe in that region, like topical Latisse. These treatments have to be tried for a couple of months before you say yea or nay, and you and the patient have to have reasonable expectations.



DN: What about men’s beards?

Dr. Hordinsky: You can treat those areas with topical or intralesional steroids. My own experience is that you have to use intralesional steroids, and overall, this form of alopecia areata may be one of the most difficult to manage successfully.



DN: Do men complain about missing chest hair?

Dr. Hordinsky: Chest hair doesn’t come up a lot for me. For men, it’s mainly the beard area. But people with alopecia areata may sometimes minimize or not bring up discussion of loss of hair in the underarms, the genital region, or the chest. It may be because they’ve figured out how to deal with it.



DN: How do you think treatments will improve over the next 5-10 years?

Dr. Hordinsky: We have a number of companies putting JAK inhibitors into clinical trials. A major step forward will be figuring out which one works the best, and then the next hurdle will be sustainability. There are very few studies in alopecia areata about how long a response to treatment can be maintained. Another big step will be the development of a topical agent that is able to penetrate through the skin to the level of the immune attack at the lower part of the hair follicle and provide the opportunity to possibly not only grow hair but also to maintain hair growth. So there’s a lot of evolution going on right now.

Dr. Hordinsky disclosed consulting work with Procter & Gamble, Concert, and Cassiopea, and grant/research support from Aclaris, National Alopecia Areata Foundation, Allergan.

SDEF and this news organization are owned by the same parent company.

LAS VEGAS – Alopecia is hard to bear for patients and has been difficult to treat, but “there’s been a revolution in thinking about how to treat alopecia areata,” according to Maria Hordinsky, MD.

Dr. Hordinsky, professor and chair of the department of dermatology, University of Minnesota, Minneapolis, discussed hair disorders in multiple presentations at Skin Disease Education Foundation’s annual Las Vegas Dermatology Seminar. In an interview after her session on alopecia areata, she elaborated on the state of research and treatment for patients with this diagnosis.



DERMATOLOGY NEWS: What has changed in alopecia areata treatment over the last few years?

Dr. Hordinsky: There is still no Food and Drug Administration–approved treatment for this disease. But recent studies have helped us understand how alopecia areata occurs, how important interleukin-15 is, and how to target this cytokine as well as others. At the same time, expertise has developed at several centers across the United States with using the Janus kinase inhibitor tofacitinib [Xeljanz] at 5 milligrams twice a day. This has led to more off-label use. Concurrently, many pharmaceutical companies with interest in alopecia areata have begun to study different JAK inhibitors that target different cytokine receptors. Researchers also are exploring topical JAK inhibitors.



DN: What about the high costs of these drugs?

Dr. Hordinsky: Some insurers are covering tofacitinib, and patient assistance programs are very helpful and beneficial. In my own practice, most patients taking these drugs are using those programs.



DN: Are any treatments being used less than in the past?

Dr. Hordinsky: We’re still using tools that we have in the toolbox because we don’t have an approved treatment. We use topical steroids and intralesional steroids. We also use prednisone as needed, and we use contact sensitization therapy.



DN: In your presentation, you talked about alopecia areata in body areas outside of the scalp. What should dermatologists know about the eyebrows in patients with alopecia areata?

Dr. Hordinsky: Some patients don’t care as much about the scalp hair loss because they’ve figured out how to deal with it. What really bothers some patients is their eyebrow hair loss. You can think of situations where alopecia areata creates a circle in the middle of the eyebrow on the left side, but not the right, or you lose one eyebrow but not the other. We use techniques such as intralesional steroids. If there’s some hair growth present that’s lightly pigmented, we may apply topical minoxidil or Latisse [bimatoprost] to the brow area. Patients may also do microblading.



DN: Are there eyebrow prosthetics?

Dr. Hordinsky: Yes, there are. The National Alopecia Areata Foundation provides a lot of information to patients and providers about these devices. There are devices that you can tape on your brow area. Some don’t look great cosmetically, but some look fantastic. Microblading may create the most normal appearance.



DN: What about eyelashes?

Dr. Hordinsky: Eyelash loss is tough and really bothersome to patients if they don’t wear glasses because of the protection provided by eyelashes, such as when you blink against airborne dust. If there is some hair present in the eyebrow regions, one can try to regrow hair and use something that’s safe in that region, like topical Latisse. These treatments have to be tried for a couple of months before you say yea or nay, and you and the patient have to have reasonable expectations.



DN: What about men’s beards?

Dr. Hordinsky: You can treat those areas with topical or intralesional steroids. My own experience is that you have to use intralesional steroids, and overall, this form of alopecia areata may be one of the most difficult to manage successfully.



DN: Do men complain about missing chest hair?

Dr. Hordinsky: Chest hair doesn’t come up a lot for me. For men, it’s mainly the beard area. But people with alopecia areata may sometimes minimize or not bring up discussion of loss of hair in the underarms, the genital region, or the chest. It may be because they’ve figured out how to deal with it.



DN: How do you think treatments will improve over the next 5-10 years?

Dr. Hordinsky: We have a number of companies putting JAK inhibitors into clinical trials. A major step forward will be figuring out which one works the best, and then the next hurdle will be sustainability. There are very few studies in alopecia areata about how long a response to treatment can be maintained. Another big step will be the development of a topical agent that is able to penetrate through the skin to the level of the immune attack at the lower part of the hair follicle and provide the opportunity to possibly not only grow hair but also to maintain hair growth. So there’s a lot of evolution going on right now.

Dr. Hordinsky disclosed consulting work with Procter & Gamble, Concert, and Cassiopea, and grant/research support from Aclaris, National Alopecia Areata Foundation, Allergan.

SDEF and this news organization are owned by the same parent company.

Pilonidal disease was first described by Mayo¹ in 1833 who hypothesized that the underlying etiology is incomplete separation of the mesoderm and ectoderm layers during embryogenesis. In 1880, Hodges² coined the term pilonidal sinus; he postulated that sinus formation was incited by hair.² Today, Hodges theory is known as the acquired theory: hair induces a foreign body response in surrounding tissue, leading to sinus formation. Although pilonidal cysts can occur anywhere on the body, they most commonly extend cephalad in the sacrococcygeal and upper gluteal cleft (Figure 1).^3,4 An acute pilonidal cyst typically presents with pain, tenderness, and swelling, similar to the presentation of a superficial abscess in other locations; however, a clue to the diagnosis is the presence of cutaneous pits along the midline of the gluteal cleft.⁵ Chronic pilonidal disease varies based on the extent of inflammation and scarring; the underlying cavity communicates with the overlying skin through sinuses and often drains with pressure.⁶

Pilonidal sinuses are rare before puberty or after 40 years of age⁷ and occur primarily in hirsute men. The ratio of men to women affected is between 3:1 and 4:1.⁸ Although pilonidal sinuses account for only 15% of anal suppurations, complications arising from pilonidal sinuses are a considerable cause of morbidity, resulting in loss of productivity in otherwise healthy individuals.⁹ Complications include chronic nonhealing wounds,¹⁰ as recurrent pilonidal sinuses tend to become colonized with gram-positive and facultative anaerobic bacteria, whereas primary pilonidal cysts more commonly become infected with anaerobic and gram-negative bacteria.¹¹ Long-standing disease increases the risk of squamous cell carcinoma arising within sinus tracts.^10,12

Histopathologically, pilonidal cysts are not true cysts because they lack an epithelial lining. Examination of the cavity commonly reveals hair, debris, and granulation tissue with surrounding foreign-body giant cells (Figure 2).⁵

The preferred treatment of pilonidal cysts continues to be debated. In this article, we review evidence supporting current modalities including conservative and surgical techniques as well as novel laser therapy for the treatment of pilonidal disease.

Conservative Management Techniques

Phenol Injections
Liquid or crystallized phenol injections have been used for treatment of mild to moderate pilonidal cysts.¹³ Excess debris is removed by curettage, and phenol is administered through the existing orifices or pits without pressure. The phenol remains in the cavity for 1 to 3 minutes before aspiration. Remaining cyst contents are removed through tissue manipulation, and the sinus is washed with saline. Mean healing time is 20 days (range, +/−14 days).¹³

Classically, phenol injections have a failure rate of 30% to 40%, especially with multiple sinuses and suppurative disease⁶; however, the success rate improves with limited disease (ie, no more than 1–3 sinus pits).³ With multiple treatment sessions, a recurrence rate as low as 2% over 25 months has been reported.¹⁴ Phenol injection also has been proposed as an adjuvant therapy to pit excision to minimize the need for extensive surgery.¹⁵

Simple Incision and Drainage
Simple incision and drainage has a crucial role in the treatment of acute pilonidal disease to decrease pain and relieve tension. Off-midline incisions have been recommended for because the resulting closures fared better against sheer forces applied by the gluteal muscles on the cleft.⁶ Therefore, the incision often is made off-midline from the gluteal cleft even when the cyst lies directly on the gluteal cleft.

Rates of healing vary widely after incision and drainage, ranging from 45% to 82%.⁶ Primary pilonidal cysts may respond well, particularly if the cavity is abraded; in one series, 79% (58/73) of patients did not have a recurrence at the average follow-up of 60 months.¹⁶

Excision and Unroofing
Techniques for excision and unroofing without primary closure include 2 variants: wide and limited. The wide technique consists of an inwardly slanted excision that is deepest in the center of the cavity. The inward sloping angle of the incision aids in healing because it allows granulation to progress evenly from the base of the wound upward. The depth of the incision should spare the fascia and leave as much fatty tissue as possible while still resecting the entire cavity and associated pits.⁶ Limited incision techniques aim to shorten the healing period by making smaller incisions into the sinuses, pits, and secondary tracts, and they are frequently supplemented with curettage.⁶ Noteworthy disadvantages include prolonged healing time, need for professional wound management, and extended medical observation.⁵ The average duration of wound healing in a study of 300 patients was 5.4 weeks (range, +/−1.1 weeks),¹⁷ and the recurrence rate has ranged from 5% to 13%.^18,19 Care must be taken to respond to numerous possible complications, including excessive exudation and granulation, superinfection, and walling off.⁶

Although the cost of treatment varies by hospital, location, and a patient’s insurance coverage, patient reports to the Pilonidal Support Alliance indicate that the cost of conservative management ranges from $500 to $2000.²⁰

Excision and Primary Closure
An elliptical excision that includes some of the lateral margin is excised down to the level of the fascia. Adjacent lateral tracts may be excised by expanding the incision. To close the wound, edges are approximated with placement of deep and superficial sutures. Wound healing typically occurs faster than secondary granulation, as seen in one randomized controlled trial with a mean of 10 days for primary closure compared to 13 weeks for secondary intention.²¹ However, as with any surgical procedure, postoperative complications can delay wound healing.¹⁹ The recurrence rate after primary closure varies considerably, ranging from 10% to 38%.^18,21-23 The average cost of an excision ranges from $3000 to $6000.²⁰

A Cochrane review evaluated 26 studies comparing primary and secondary closure. This large analysis showed no clear benefit for open healing over surgical closure²⁴; however, off-midline closure showed statistically significant benefit over midline closure (mean difference, 5.4 days; 95% CI, 2.3-8.5), and many experts now consider off-midline closure the standard of care in pilonidal sinus management (Figure 3).^24,25

Surgical Techniques

For severe or recurrent pilonidal disease, skin flaps often are required. Several flaps have been developed, including advancement, Bascom cleft lift, Karydakis, and modified Limberg flap. Flaps require a vascular pedicle but allow for closure without tension.²⁶ The cost of a flap procedure, ranging from $10,000 to $30,000, is greater than the cost of excision or other conservative therapy²⁰; however, with a lower recurrence rate of pilonidal disease following flap procedures compared to other treatments, patients may save more on treatment over the long-term.

Advancement Flaps
The most commonly used advancement flaps are the V-Y advancement flap and Z-plasty. The V-Y advancement flap creates a full-thickness V-shaped incision down to gluteal fascia that is closed to form a postrepair suture line in the shape of a Y.⁵ Depending on the size of the defect, the flaps may be utilized unilaterally or bilaterally. A defect as large as 8 to 10 cm can be covered unilaterally; however, defects larger than 10 cm commonly require a bilateral flap.²⁶ The V-Y advancement flap failed to show superiority to primary closure techniques based on complications, recurrence, and patient satisfaction in a large randomized controlled trial.²⁷

Performing a Z-plasty requires excision of diseased tissue with recruitment of lateral flaps incised down to the level of the fascia. The lateral edges are transposed to increase transverse length.²⁶ No statistically significant difference in infection or recurrence rates was noted between excision alone and excision plus Z-plasty; however, wounds were reported to heal faster in patients receiving excision plus Z-plasty (41 vs 15 days).²⁸

Cleft Lift Closure
In 1987, Bascom²⁹ introduced the cleft lift closure for recurrent pilonidal disease. This technique aims to reduce or eliminate lateral gluteal forces on the wounds by filling the gluteal cleft.⁵ The sinus tracts are excised and a full-thickness skin flap is extended across the cleft and closed off-midline. The adipose tissue fills in the previous space of the gluteal cleft. In the initial study, no recurrences were reported in 30 patients who underwent this procedure at 2-year follow-up; similarly, in another case series of 26 patients who underwent the procedure, no recurrences were noted at a median follow-up of 3 years.³⁰ Compared to excision with secondary wound healing and primary closure on the midline, the Bascom cleft lift demonstrated a decrease in wound healing time (62, 52, and 29 days, respectively).³¹

The classic Karydakis flap consists of an oblique elliptical excision of diseased tissue with fixation of the flap base to the sacral fascia (Figures 4 and 5). The flap is closed by suturing the edge off-midline.³² This technique prevents a midline wound and aims to remodel and flatten the natal cleft. Karydakis³³ performed the most important study for treatment of pilonidal disease with the Karydakis flap, which included more than 5000 patients. The results showed a 0.9% recurrence rate and an 8.5% wound complication rate over a 2- to 20-year follow-up.³³ These results have been substantiated by more recent studies, which produced similar results: a 1.8% to 5.3% infection rate and a recurrence rate of 0.9% to 4.4%.^34,35

In the modified Karydakis flap, the same excision and closure is performed without tacking the flap to the sacral fascia, aiming to prevent formation of a new vulnerable raphe by flattening the natal cleft. The infection rate was similar to the classic Karydakis flap, and no recurrences were noted during a 20-month follow-up.³⁶

Limberg Flap
The Limberg flap is derived from a rhomboid flap. In the classic Limberg flap, a midline rhomboid incision to the presacral fascia including the sinus is performed. The flap gains mobility by extending the excision laterally to the fascia of the gluteus maximus muscle. A variant of the original flap includes the modified Limberg flap, which lateralizes the midline sutures and flattens the intergluteal sulcus. Compared to the traditional Limberg approach, the modified Limberg flap was associated with a lower failure rate at both early and late time points and a lower rate of infection^37,38; however, based on the data it is unclear when primary closure should be favored over a Limberg flap. Several studies show the recurrence rate to be identical; however, hospital stay and pain were reduced in the Limberg flap group compared to primary closure.^39,40

Results from randomized controlled trials comparing the modified Limberg flap to the Karydakis flap vary. One of the largest prospective, randomized, controlled trials comparing the 2 flaps included 269 patients.Results showed a lower postoperative complication rate, lower pain scores, shorter operation time, and shorter hospital stay with the Karydakis flap compared to the Limberg flap, though no difference in recurrence was noted between the 2 groups.⁴¹

Two randomized controlled trials comprising 145 and 120 patients, respectively, showed no statistically significant difference between the Limberg flap and Karydakis flap with regard to complication rate, length of stay, and recurrence rate^36,42; however, patients in the Karydakis group reported subjectively feeling healed more quickly than patients in the modified Limberg flap group,⁴² and 1 of the 2 studies showed an increase in patient satisfaction with the modified Karydakis flap compared to modified Limberg flap.³⁶ In contrast to earlier studies, a 2009 study showed the Karydakis flap was associated with a higher wound infection rate than the Limberg flap group in a randomized trial of 100 patients (13/50 vs 4/50 patients).⁴³

Overall, larger prospective trials are needed to clarify the differences in outcomes between flap techniques. In our opinion, variations in postoperative complication and recurrence rates likely are due to differences in surgeon comfort and surgical technique. The Table provides a comprehensive list of trials comparing flap techniques.

Laser Therapy

Lasers are emerging as primary and adjuvant treatment options for pilonidal sinuses. Depilation with alexandrite, diode, and Nd:YAG lasers has demonstrated the most consistent evidence.^50-54 The firm texture and quality of the hair is proposed to incite an inflammatory response with sinus formation; therefore, using a laser to permaently remove this factor may help prevent future disease.

Large randomized controlled trials are needed to fully determine the utility of laser therapy as a primary or adjuvant treatment in pilonidal disease; however, given that laser therapies address the core pathogenesis of pilonidal disease and generally are well tolerated, their use may be strongly considered.

Conclusion

With mild pilonidal disease, more conservative measures can be employed; however, in cases of recurrent or suppurative disease or extensive scarring, excision with flap closure typically is required. Although no single surgical procedure has been identified as superior, one review demonstrated that off-midline procedures are statistically superior to midline closure in healing time, surgical site infection, and recurrence rate.²⁴ Novel techniques continue to emerge in the management of pilonidal disease, including laser therapy. This modality shows promise as either a primary or adjuvant treatment; however, large randomized controlled trials are needed to confirm early findings.

Given that pilonidal disease most commonly occurs in the actively employed population, we recommend that dermatologic surgeons discuss treatment options with patients who have pilonidal disease, taking into consideration cost, length of hospital stay, and recovery time when deciding on a treatment course.

Pilonidal disease was first described by Mayo¹ in 1833 who hypothesized that the underlying etiology is incomplete separation of the mesoderm and ectoderm layers during embryogenesis. In 1880, Hodges² coined the term pilonidal sinus; he postulated that sinus formation was incited by hair.² Today, Hodges theory is known as the acquired theory: hair induces a foreign body response in surrounding tissue, leading to sinus formation. Although pilonidal cysts can occur anywhere on the body, they most commonly extend cephalad in the sacrococcygeal and upper gluteal cleft (Figure 1).^3,4 An acute pilonidal cyst typically presents with pain, tenderness, and swelling, similar to the presentation of a superficial abscess in other locations; however, a clue to the diagnosis is the presence of cutaneous pits along the midline of the gluteal cleft.⁵ Chronic pilonidal disease varies based on the extent of inflammation and scarring; the underlying cavity communicates with the overlying skin through sinuses and often drains with pressure.⁶

Pilonidal sinuses are rare before puberty or after 40 years of age⁷ and occur primarily in hirsute men. The ratio of men to women affected is between 3:1 and 4:1.⁸ Although pilonidal sinuses account for only 15% of anal suppurations, complications arising from pilonidal sinuses are a considerable cause of morbidity, resulting in loss of productivity in otherwise healthy individuals.⁹ Complications include chronic nonhealing wounds,¹⁰ as recurrent pilonidal sinuses tend to become colonized with gram-positive and facultative anaerobic bacteria, whereas primary pilonidal cysts more commonly become infected with anaerobic and gram-negative bacteria.¹¹ Long-standing disease increases the risk of squamous cell carcinoma arising within sinus tracts.^10,12

Histopathologically, pilonidal cysts are not true cysts because they lack an epithelial lining. Examination of the cavity commonly reveals hair, debris, and granulation tissue with surrounding foreign-body giant cells (Figure 2).⁵

The preferred treatment of pilonidal cysts continues to be debated. In this article, we review evidence supporting current modalities including conservative and surgical techniques as well as novel laser therapy for the treatment of pilonidal disease.

Conservative Management Techniques

Phenol Injections
Liquid or crystallized phenol injections have been used for treatment of mild to moderate pilonidal cysts.¹³ Excess debris is removed by curettage, and phenol is administered through the existing orifices or pits without pressure. The phenol remains in the cavity for 1 to 3 minutes before aspiration. Remaining cyst contents are removed through tissue manipulation, and the sinus is washed with saline. Mean healing time is 20 days (range, +/−14 days).¹³

Classically, phenol injections have a failure rate of 30% to 40%, especially with multiple sinuses and suppurative disease⁶; however, the success rate improves with limited disease (ie, no more than 1–3 sinus pits).³ With multiple treatment sessions, a recurrence rate as low as 2% over 25 months has been reported.¹⁴ Phenol injection also has been proposed as an adjuvant therapy to pit excision to minimize the need for extensive surgery.¹⁵

Simple Incision and Drainage
Simple incision and drainage has a crucial role in the treatment of acute pilonidal disease to decrease pain and relieve tension. Off-midline incisions have been recommended for because the resulting closures fared better against sheer forces applied by the gluteal muscles on the cleft.⁶ Therefore, the incision often is made off-midline from the gluteal cleft even when the cyst lies directly on the gluteal cleft.

Rates of healing vary widely after incision and drainage, ranging from 45% to 82%.⁶ Primary pilonidal cysts may respond well, particularly if the cavity is abraded; in one series, 79% (58/73) of patients did not have a recurrence at the average follow-up of 60 months.¹⁶

Excision and Unroofing
Techniques for excision and unroofing without primary closure include 2 variants: wide and limited. The wide technique consists of an inwardly slanted excision that is deepest in the center of the cavity. The inward sloping angle of the incision aids in healing because it allows granulation to progress evenly from the base of the wound upward. The depth of the incision should spare the fascia and leave as much fatty tissue as possible while still resecting the entire cavity and associated pits.⁶ Limited incision techniques aim to shorten the healing period by making smaller incisions into the sinuses, pits, and secondary tracts, and they are frequently supplemented with curettage.⁶ Noteworthy disadvantages include prolonged healing time, need for professional wound management, and extended medical observation.⁵ The average duration of wound healing in a study of 300 patients was 5.4 weeks (range, +/−1.1 weeks),¹⁷ and the recurrence rate has ranged from 5% to 13%.^18,19 Care must be taken to respond to numerous possible complications, including excessive exudation and granulation, superinfection, and walling off.⁶

Although the cost of treatment varies by hospital, location, and a patient’s insurance coverage, patient reports to the Pilonidal Support Alliance indicate that the cost of conservative management ranges from $500 to $2000.²⁰

Excision and Primary Closure
An elliptical excision that includes some of the lateral margin is excised down to the level of the fascia. Adjacent lateral tracts may be excised by expanding the incision. To close the wound, edges are approximated with placement of deep and superficial sutures. Wound healing typically occurs faster than secondary granulation, as seen in one randomized controlled trial with a mean of 10 days for primary closure compared to 13 weeks for secondary intention.²¹ However, as with any surgical procedure, postoperative complications can delay wound healing.¹⁹ The recurrence rate after primary closure varies considerably, ranging from 10% to 38%.^18,21-23 The average cost of an excision ranges from $3000 to $6000.²⁰

A Cochrane review evaluated 26 studies comparing primary and secondary closure. This large analysis showed no clear benefit for open healing over surgical closure²⁴; however, off-midline closure showed statistically significant benefit over midline closure (mean difference, 5.4 days; 95% CI, 2.3-8.5), and many experts now consider off-midline closure the standard of care in pilonidal sinus management (Figure 3).^24,25

Surgical Techniques

For severe or recurrent pilonidal disease, skin flaps often are required. Several flaps have been developed, including advancement, Bascom cleft lift, Karydakis, and modified Limberg flap. Flaps require a vascular pedicle but allow for closure without tension.²⁶ The cost of a flap procedure, ranging from $10,000 to $30,000, is greater than the cost of excision or other conservative therapy²⁰; however, with a lower recurrence rate of pilonidal disease following flap procedures compared to other treatments, patients may save more on treatment over the long-term.

Advancement Flaps
The most commonly used advancement flaps are the V-Y advancement flap and Z-plasty. The V-Y advancement flap creates a full-thickness V-shaped incision down to gluteal fascia that is closed to form a postrepair suture line in the shape of a Y.⁵ Depending on the size of the defect, the flaps may be utilized unilaterally or bilaterally. A defect as large as 8 to 10 cm can be covered unilaterally; however, defects larger than 10 cm commonly require a bilateral flap.²⁶ The V-Y advancement flap failed to show superiority to primary closure techniques based on complications, recurrence, and patient satisfaction in a large randomized controlled trial.²⁷

Performing a Z-plasty requires excision of diseased tissue with recruitment of lateral flaps incised down to the level of the fascia. The lateral edges are transposed to increase transverse length.²⁶ No statistically significant difference in infection or recurrence rates was noted between excision alone and excision plus Z-plasty; however, wounds were reported to heal faster in patients receiving excision plus Z-plasty (41 vs 15 days).²⁸

Cleft Lift Closure
In 1987, Bascom²⁹ introduced the cleft lift closure for recurrent pilonidal disease. This technique aims to reduce or eliminate lateral gluteal forces on the wounds by filling the gluteal cleft.⁵ The sinus tracts are excised and a full-thickness skin flap is extended across the cleft and closed off-midline. The adipose tissue fills in the previous space of the gluteal cleft. In the initial study, no recurrences were reported in 30 patients who underwent this procedure at 2-year follow-up; similarly, in another case series of 26 patients who underwent the procedure, no recurrences were noted at a median follow-up of 3 years.³⁰ Compared to excision with secondary wound healing and primary closure on the midline, the Bascom cleft lift demonstrated a decrease in wound healing time (62, 52, and 29 days, respectively).³¹

The classic Karydakis flap consists of an oblique elliptical excision of diseased tissue with fixation of the flap base to the sacral fascia (Figures 4 and 5). The flap is closed by suturing the edge off-midline.³² This technique prevents a midline wound and aims to remodel and flatten the natal cleft. Karydakis³³ performed the most important study for treatment of pilonidal disease with the Karydakis flap, which included more than 5000 patients. The results showed a 0.9% recurrence rate and an 8.5% wound complication rate over a 2- to 20-year follow-up.³³ These results have been substantiated by more recent studies, which produced similar results: a 1.8% to 5.3% infection rate and a recurrence rate of 0.9% to 4.4%.^34,35

In the modified Karydakis flap, the same excision and closure is performed without tacking the flap to the sacral fascia, aiming to prevent formation of a new vulnerable raphe by flattening the natal cleft. The infection rate was similar to the classic Karydakis flap, and no recurrences were noted during a 20-month follow-up.³⁶

Limberg Flap
The Limberg flap is derived from a rhomboid flap. In the classic Limberg flap, a midline rhomboid incision to the presacral fascia including the sinus is performed. The flap gains mobility by extending the excision laterally to the fascia of the gluteus maximus muscle. A variant of the original flap includes the modified Limberg flap, which lateralizes the midline sutures and flattens the intergluteal sulcus. Compared to the traditional Limberg approach, the modified Limberg flap was associated with a lower failure rate at both early and late time points and a lower rate of infection^37,38; however, based on the data it is unclear when primary closure should be favored over a Limberg flap. Several studies show the recurrence rate to be identical; however, hospital stay and pain were reduced in the Limberg flap group compared to primary closure.^39,40

Results from randomized controlled trials comparing the modified Limberg flap to the Karydakis flap vary. One of the largest prospective, randomized, controlled trials comparing the 2 flaps included 269 patients.Results showed a lower postoperative complication rate, lower pain scores, shorter operation time, and shorter hospital stay with the Karydakis flap compared to the Limberg flap, though no difference in recurrence was noted between the 2 groups.⁴¹

Two randomized controlled trials comprising 145 and 120 patients, respectively, showed no statistically significant difference between the Limberg flap and Karydakis flap with regard to complication rate, length of stay, and recurrence rate^36,42; however, patients in the Karydakis group reported subjectively feeling healed more quickly than patients in the modified Limberg flap group,⁴² and 1 of the 2 studies showed an increase in patient satisfaction with the modified Karydakis flap compared to modified Limberg flap.³⁶ In contrast to earlier studies, a 2009 study showed the Karydakis flap was associated with a higher wound infection rate than the Limberg flap group in a randomized trial of 100 patients (13/50 vs 4/50 patients).⁴³

Overall, larger prospective trials are needed to clarify the differences in outcomes between flap techniques. In our opinion, variations in postoperative complication and recurrence rates likely are due to differences in surgeon comfort and surgical technique. The Table provides a comprehensive list of trials comparing flap techniques.

Laser Therapy

Lasers are emerging as primary and adjuvant treatment options for pilonidal sinuses. Depilation with alexandrite, diode, and Nd:YAG lasers has demonstrated the most consistent evidence.^50-54 The firm texture and quality of the hair is proposed to incite an inflammatory response with sinus formation; therefore, using a laser to permaently remove this factor may help prevent future disease.

Large randomized controlled trials are needed to fully determine the utility of laser therapy as a primary or adjuvant treatment in pilonidal disease; however, given that laser therapies address the core pathogenesis of pilonidal disease and generally are well tolerated, their use may be strongly considered.

Conclusion

With mild pilonidal disease, more conservative measures can be employed; however, in cases of recurrent or suppurative disease or extensive scarring, excision with flap closure typically is required. Although no single surgical procedure has been identified as superior, one review demonstrated that off-midline procedures are statistically superior to midline closure in healing time, surgical site infection, and recurrence rate.²⁴ Novel techniques continue to emerge in the management of pilonidal disease, including laser therapy. This modality shows promise as either a primary or adjuvant treatment; however, large randomized controlled trials are needed to confirm early findings.

Given that pilonidal disease most commonly occurs in the actively employed population, we recommend that dermatologic surgeons discuss treatment options with patients who have pilonidal disease, taking into consideration cost, length of hospital stay, and recovery time when deciding on a treatment course.

Pilonidal disease was first described by Mayo¹ in 1833 who hypothesized that the underlying etiology is incomplete separation of the mesoderm and ectoderm layers during embryogenesis. In 1880, Hodges² coined the term pilonidal sinus; he postulated that sinus formation was incited by hair.² Today, Hodges theory is known as the acquired theory: hair induces a foreign body response in surrounding tissue, leading to sinus formation. Although pilonidal cysts can occur anywhere on the body, they most commonly extend cephalad in the sacrococcygeal and upper gluteal cleft (Figure 1).^3,4 An acute pilonidal cyst typically presents with pain, tenderness, and swelling, similar to the presentation of a superficial abscess in other locations; however, a clue to the diagnosis is the presence of cutaneous pits along the midline of the gluteal cleft.⁵ Chronic pilonidal disease varies based on the extent of inflammation and scarring; the underlying cavity communicates with the overlying skin through sinuses and often drains with pressure.⁶

Pilonidal sinuses are rare before puberty or after 40 years of age⁷ and occur primarily in hirsute men. The ratio of men to women affected is between 3:1 and 4:1.⁸ Although pilonidal sinuses account for only 15% of anal suppurations, complications arising from pilonidal sinuses are a considerable cause of morbidity, resulting in loss of productivity in otherwise healthy individuals.⁹ Complications include chronic nonhealing wounds,¹⁰ as recurrent pilonidal sinuses tend to become colonized with gram-positive and facultative anaerobic bacteria, whereas primary pilonidal cysts more commonly become infected with anaerobic and gram-negative bacteria.¹¹ Long-standing disease increases the risk of squamous cell carcinoma arising within sinus tracts.^10,12

Histopathologically, pilonidal cysts are not true cysts because they lack an epithelial lining. Examination of the cavity commonly reveals hair, debris, and granulation tissue with surrounding foreign-body giant cells (Figure 2).⁵

The preferred treatment of pilonidal cysts continues to be debated. In this article, we review evidence supporting current modalities including conservative and surgical techniques as well as novel laser therapy for the treatment of pilonidal disease.

Conservative Management Techniques

Phenol Injections
Liquid or crystallized phenol injections have been used for treatment of mild to moderate pilonidal cysts.¹³ Excess debris is removed by curettage, and phenol is administered through the existing orifices or pits without pressure. The phenol remains in the cavity for 1 to 3 minutes before aspiration. Remaining cyst contents are removed through tissue manipulation, and the sinus is washed with saline. Mean healing time is 20 days (range, +/−14 days).¹³

Classically, phenol injections have a failure rate of 30% to 40%, especially with multiple sinuses and suppurative disease⁶; however, the success rate improves with limited disease (ie, no more than 1–3 sinus pits).³ With multiple treatment sessions, a recurrence rate as low as 2% over 25 months has been reported.¹⁴ Phenol injection also has been proposed as an adjuvant therapy to pit excision to minimize the need for extensive surgery.¹⁵

Simple Incision and Drainage
Simple incision and drainage has a crucial role in the treatment of acute pilonidal disease to decrease pain and relieve tension. Off-midline incisions have been recommended for because the resulting closures fared better against sheer forces applied by the gluteal muscles on the cleft.⁶ Therefore, the incision often is made off-midline from the gluteal cleft even when the cyst lies directly on the gluteal cleft.

Rates of healing vary widely after incision and drainage, ranging from 45% to 82%.⁶ Primary pilonidal cysts may respond well, particularly if the cavity is abraded; in one series, 79% (58/73) of patients did not have a recurrence at the average follow-up of 60 months.¹⁶

Excision and Unroofing
Techniques for excision and unroofing without primary closure include 2 variants: wide and limited. The wide technique consists of an inwardly slanted excision that is deepest in the center of the cavity. The inward sloping angle of the incision aids in healing because it allows granulation to progress evenly from the base of the wound upward. The depth of the incision should spare the fascia and leave as much fatty tissue as possible while still resecting the entire cavity and associated pits.⁶ Limited incision techniques aim to shorten the healing period by making smaller incisions into the sinuses, pits, and secondary tracts, and they are frequently supplemented with curettage.⁶ Noteworthy disadvantages include prolonged healing time, need for professional wound management, and extended medical observation.⁵ The average duration of wound healing in a study of 300 patients was 5.4 weeks (range, +/−1.1 weeks),¹⁷ and the recurrence rate has ranged from 5% to 13%.^18,19 Care must be taken to respond to numerous possible complications, including excessive exudation and granulation, superinfection, and walling off.⁶

Although the cost of treatment varies by hospital, location, and a patient’s insurance coverage, patient reports to the Pilonidal Support Alliance indicate that the cost of conservative management ranges from $500 to $2000.²⁰

Excision and Primary Closure
An elliptical excision that includes some of the lateral margin is excised down to the level of the fascia. Adjacent lateral tracts may be excised by expanding the incision. To close the wound, edges are approximated with placement of deep and superficial sutures. Wound healing typically occurs faster than secondary granulation, as seen in one randomized controlled trial with a mean of 10 days for primary closure compared to 13 weeks for secondary intention.²¹ However, as with any surgical procedure, postoperative complications can delay wound healing.¹⁹ The recurrence rate after primary closure varies considerably, ranging from 10% to 38%.^18,21-23 The average cost of an excision ranges from $3000 to $6000.²⁰

A Cochrane review evaluated 26 studies comparing primary and secondary closure. This large analysis showed no clear benefit for open healing over surgical closure²⁴; however, off-midline closure showed statistically significant benefit over midline closure (mean difference, 5.4 days; 95% CI, 2.3-8.5), and many experts now consider off-midline closure the standard of care in pilonidal sinus management (Figure 3).^24,25

Surgical Techniques

For severe or recurrent pilonidal disease, skin flaps often are required. Several flaps have been developed, including advancement, Bascom cleft lift, Karydakis, and modified Limberg flap. Flaps require a vascular pedicle but allow for closure without tension.²⁶ The cost of a flap procedure, ranging from $10,000 to $30,000, is greater than the cost of excision or other conservative therapy²⁰; however, with a lower recurrence rate of pilonidal disease following flap procedures compared to other treatments, patients may save more on treatment over the long-term.

Advancement Flaps
The most commonly used advancement flaps are the V-Y advancement flap and Z-plasty. The V-Y advancement flap creates a full-thickness V-shaped incision down to gluteal fascia that is closed to form a postrepair suture line in the shape of a Y.⁵ Depending on the size of the defect, the flaps may be utilized unilaterally or bilaterally. A defect as large as 8 to 10 cm can be covered unilaterally; however, defects larger than 10 cm commonly require a bilateral flap.²⁶ The V-Y advancement flap failed to show superiority to primary closure techniques based on complications, recurrence, and patient satisfaction in a large randomized controlled trial.²⁷

Performing a Z-plasty requires excision of diseased tissue with recruitment of lateral flaps incised down to the level of the fascia. The lateral edges are transposed to increase transverse length.²⁶ No statistically significant difference in infection or recurrence rates was noted between excision alone and excision plus Z-plasty; however, wounds were reported to heal faster in patients receiving excision plus Z-plasty (41 vs 15 days).²⁸

Cleft Lift Closure
In 1987, Bascom²⁹ introduced the cleft lift closure for recurrent pilonidal disease. This technique aims to reduce or eliminate lateral gluteal forces on the wounds by filling the gluteal cleft.⁵ The sinus tracts are excised and a full-thickness skin flap is extended across the cleft and closed off-midline. The adipose tissue fills in the previous space of the gluteal cleft. In the initial study, no recurrences were reported in 30 patients who underwent this procedure at 2-year follow-up; similarly, in another case series of 26 patients who underwent the procedure, no recurrences were noted at a median follow-up of 3 years.³⁰ Compared to excision with secondary wound healing and primary closure on the midline, the Bascom cleft lift demonstrated a decrease in wound healing time (62, 52, and 29 days, respectively).³¹

The classic Karydakis flap consists of an oblique elliptical excision of diseased tissue with fixation of the flap base to the sacral fascia (Figures 4 and 5). The flap is closed by suturing the edge off-midline.³² This technique prevents a midline wound and aims to remodel and flatten the natal cleft. Karydakis³³ performed the most important study for treatment of pilonidal disease with the Karydakis flap, which included more than 5000 patients. The results showed a 0.9% recurrence rate and an 8.5% wound complication rate over a 2- to 20-year follow-up.³³ These results have been substantiated by more recent studies, which produced similar results: a 1.8% to 5.3% infection rate and a recurrence rate of 0.9% to 4.4%.^34,35

In the modified Karydakis flap, the same excision and closure is performed without tacking the flap to the sacral fascia, aiming to prevent formation of a new vulnerable raphe by flattening the natal cleft. The infection rate was similar to the classic Karydakis flap, and no recurrences were noted during a 20-month follow-up.³⁶

Limberg Flap
The Limberg flap is derived from a rhomboid flap. In the classic Limberg flap, a midline rhomboid incision to the presacral fascia including the sinus is performed. The flap gains mobility by extending the excision laterally to the fascia of the gluteus maximus muscle. A variant of the original flap includes the modified Limberg flap, which lateralizes the midline sutures and flattens the intergluteal sulcus. Compared to the traditional Limberg approach, the modified Limberg flap was associated with a lower failure rate at both early and late time points and a lower rate of infection^37,38; however, based on the data it is unclear when primary closure should be favored over a Limberg flap. Several studies show the recurrence rate to be identical; however, hospital stay and pain were reduced in the Limberg flap group compared to primary closure.^39,40

Results from randomized controlled trials comparing the modified Limberg flap to the Karydakis flap vary. One of the largest prospective, randomized, controlled trials comparing the 2 flaps included 269 patients.Results showed a lower postoperative complication rate, lower pain scores, shorter operation time, and shorter hospital stay with the Karydakis flap compared to the Limberg flap, though no difference in recurrence was noted between the 2 groups.⁴¹

Two randomized controlled trials comprising 145 and 120 patients, respectively, showed no statistically significant difference between the Limberg flap and Karydakis flap with regard to complication rate, length of stay, and recurrence rate^36,42; however, patients in the Karydakis group reported subjectively feeling healed more quickly than patients in the modified Limberg flap group,⁴² and 1 of the 2 studies showed an increase in patient satisfaction with the modified Karydakis flap compared to modified Limberg flap.³⁶ In contrast to earlier studies, a 2009 study showed the Karydakis flap was associated with a higher wound infection rate than the Limberg flap group in a randomized trial of 100 patients (13/50 vs 4/50 patients).⁴³

Overall, larger prospective trials are needed to clarify the differences in outcomes between flap techniques. In our opinion, variations in postoperative complication and recurrence rates likely are due to differences in surgeon comfort and surgical technique. The Table provides a comprehensive list of trials comparing flap techniques.

Laser Therapy

Lasers are emerging as primary and adjuvant treatment options for pilonidal sinuses. Depilation with alexandrite, diode, and Nd:YAG lasers has demonstrated the most consistent evidence.^50-54 The firm texture and quality of the hair is proposed to incite an inflammatory response with sinus formation; therefore, using a laser to permaently remove this factor may help prevent future disease.

Large randomized controlled trials are needed to fully determine the utility of laser therapy as a primary or adjuvant treatment in pilonidal disease; however, given that laser therapies address the core pathogenesis of pilonidal disease and generally are well tolerated, their use may be strongly considered.

Conclusion

With mild pilonidal disease, more conservative measures can be employed; however, in cases of recurrent or suppurative disease or extensive scarring, excision with flap closure typically is required. Although no single surgical procedure has been identified as superior, one review demonstrated that off-midline procedures are statistically superior to midline closure in healing time, surgical site infection, and recurrence rate.²⁴ Novel techniques continue to emerge in the management of pilonidal disease, including laser therapy. This modality shows promise as either a primary or adjuvant treatment; however, large randomized controlled trials are needed to confirm early findings.

Given that pilonidal disease most commonly occurs in the actively employed population, we recommend that dermatologic surgeons discuss treatment options with patients who have pilonidal disease, taking into consideration cost, length of hospital stay, and recovery time when deciding on a treatment course.

LAS VEGAS – Taking a detailed history is especially important when it comes to alopecia areata (AA), according to Maria Hordinsky, MD, professor and chair of the department of dermatology at the University of Minnesota, Minneapolis.

Other important steps in the work-up of a patient include extensive documentation of AA – including use of the Severity of Alopecia Tool (SALT) score or photography – and laboratory testing, Dr. Hordinsky said at the Skin Disease Education Foundation’s annual Las Vegas Dermatology Seminar.

AA affects an estimated 1.7%-2.1% of the population and is sporadic in 50%-80% of cases. AA targets follicles in the anagen phase, which makes up 90% of hair follicles and normally lasts for 2-7 years. The condition has been linked to vitiligo, thyroid disease, and atopy (allergic rhinitis, asthma, and atopic dermatitis). AA has also been linked to autoimmune disorders, such as lupus erythematosus, psoriasis, rheumatoid arthritis, celiac disease, and type 1 diabetes mellitus. In addition, Dr. Hordinsky said, genetic research has linked alopecia to the latter three conditions.

It’s important to ask how much the hair loss bothers the patient, regardless of his or her age, Dr. Hordinsky said. “You need to figure out what’s making them all anxious and depressed. Is it shedding hair every single day?” she said. “Figure out what bothers that patient and go after that issue after you’ve answered his or her questions.”

In some cases, she said, “patients who’ve had extensive disease for a very long time are really coming in for a social visit to talk about what’s new, what’s emerging, whether they’d be interested in participating in a clinical trial.”

Dr. Hordinsky provided this list of questions to help in evaluating these patients:

• Is scalp hair loss the biggest problem? Eyebrow loss? Eyelash loss? Nail issues? All of these?
• How bothersome is it to experience hair shedding every day? Does this make the patient depressed and anxious?

Dr. Hordinsky added that asking patients about the following topics can also be appropriate:

• Hair care habits. Is the patient no longer shampooing because of hair loss?
• Medications.
• Symptoms, such as pain, itch, and burning.
• Body hair. Does the patient feel there’s too little or too much?
• Nail abnormalities.
• Menstrual cycle and pregnancies.
• Diet and supplements.
• Family history. Patients – who are often very curious about the disease – often mention relatives when they hear about a genetic role in AA, she said. They’ll say “My aunt has rheumatoid arthritis” or “My cousin has autoimmune disease.”
• Excess androgen levels and autoimmune/endocrine diseases.

The patient examination should include documentation of the presence of vellus hair or indeterminate and terminal fibers; the presence or absence of scale, erythema, folliculitis, and atrophy; eyebrow, eyelash, or body hair loss; and nail damage, such as pitting.

Photography and/or the SALT score may be used for documentation, she said. The SALT score is the only standardized method of assessment for AA, she said. The score is produced by a calculation that takes into account the amount of hair loss in the quadrants (right, left, front, back) of the scalp (J Am Acad Dermatol. 2018 Sep;79[3]:470-478.e3).

Laboratory tests are important, but there is no standard list, Dr. Hordinsky said. They are selected based on history and physical examination and can include thyroid function studies; heme and iron profiles, including serum ferritin and hemoglobin; and measurements of vitamin D, thiamine, zinc, and total protein. “We check nutrition labs particularly if a patient has been taking a lot of supplements, and we’re not sure where we’re at,” Dr. Hordinsky said.

Dermatologists may also order tests to measure non-cycle dependent hormones such as dehydroepiandrosterone sulfate (DHEAS), total/free testosterone, and antinuclear antibodies and other autoantibodies.

SDEF and this news organization are owned by the same parent company.

Dr. Hordinsky disclosed consulting work with Procter & Gamble, Concert, and Cassiopea and grant/research support from Aclaris, National Alopecia Areata Foundation, Allergan.

LAS VEGAS – Taking a detailed history is especially important when it comes to alopecia areata (AA), according to Maria Hordinsky, MD, professor and chair of the department of dermatology at the University of Minnesota, Minneapolis.

Other important steps in the work-up of a patient include extensive documentation of AA – including use of the Severity of Alopecia Tool (SALT) score or photography – and laboratory testing, Dr. Hordinsky said at the Skin Disease Education Foundation’s annual Las Vegas Dermatology Seminar.

AA affects an estimated 1.7%-2.1% of the population and is sporadic in 50%-80% of cases. AA targets follicles in the anagen phase, which makes up 90% of hair follicles and normally lasts for 2-7 years. The condition has been linked to vitiligo, thyroid disease, and atopy (allergic rhinitis, asthma, and atopic dermatitis). AA has also been linked to autoimmune disorders, such as lupus erythematosus, psoriasis, rheumatoid arthritis, celiac disease, and type 1 diabetes mellitus. In addition, Dr. Hordinsky said, genetic research has linked alopecia to the latter three conditions.

It’s important to ask how much the hair loss bothers the patient, regardless of his or her age, Dr. Hordinsky said. “You need to figure out what’s making them all anxious and depressed. Is it shedding hair every single day?” she said. “Figure out what bothers that patient and go after that issue after you’ve answered his or her questions.”

In some cases, she said, “patients who’ve had extensive disease for a very long time are really coming in for a social visit to talk about what’s new, what’s emerging, whether they’d be interested in participating in a clinical trial.”

Dr. Hordinsky provided this list of questions to help in evaluating these patients:

• Is scalp hair loss the biggest problem? Eyebrow loss? Eyelash loss? Nail issues? All of these?
• How bothersome is it to experience hair shedding every day? Does this make the patient depressed and anxious?

Dr. Hordinsky added that asking patients about the following topics can also be appropriate:

• Hair care habits. Is the patient no longer shampooing because of hair loss?
• Medications.
• Symptoms, such as pain, itch, and burning.
• Body hair. Does the patient feel there’s too little or too much?
• Nail abnormalities.
• Menstrual cycle and pregnancies.
• Diet and supplements.
• Family history. Patients – who are often very curious about the disease – often mention relatives when they hear about a genetic role in AA, she said. They’ll say “My aunt has rheumatoid arthritis” or “My cousin has autoimmune disease.”
• Excess androgen levels and autoimmune/endocrine diseases.

The patient examination should include documentation of the presence of vellus hair or indeterminate and terminal fibers; the presence or absence of scale, erythema, folliculitis, and atrophy; eyebrow, eyelash, or body hair loss; and nail damage, such as pitting.

Photography and/or the SALT score may be used for documentation, she said. The SALT score is the only standardized method of assessment for AA, she said. The score is produced by a calculation that takes into account the amount of hair loss in the quadrants (right, left, front, back) of the scalp (J Am Acad Dermatol. 2018 Sep;79[3]:470-478.e3).

Laboratory tests are important, but there is no standard list, Dr. Hordinsky said. They are selected based on history and physical examination and can include thyroid function studies; heme and iron profiles, including serum ferritin and hemoglobin; and measurements of vitamin D, thiamine, zinc, and total protein. “We check nutrition labs particularly if a patient has been taking a lot of supplements, and we’re not sure where we’re at,” Dr. Hordinsky said.

Dermatologists may also order tests to measure non-cycle dependent hormones such as dehydroepiandrosterone sulfate (DHEAS), total/free testosterone, and antinuclear antibodies and other autoantibodies.

SDEF and this news organization are owned by the same parent company.

Dr. Hordinsky disclosed consulting work with Procter & Gamble, Concert, and Cassiopea and grant/research support from Aclaris, National Alopecia Areata Foundation, Allergan.

LAS VEGAS – Taking a detailed history is especially important when it comes to alopecia areata (AA), according to Maria Hordinsky, MD, professor and chair of the department of dermatology at the University of Minnesota, Minneapolis.

Other important steps in the work-up of a patient include extensive documentation of AA – including use of the Severity of Alopecia Tool (SALT) score or photography – and laboratory testing, Dr. Hordinsky said at the Skin Disease Education Foundation’s annual Las Vegas Dermatology Seminar.

AA affects an estimated 1.7%-2.1% of the population and is sporadic in 50%-80% of cases. AA targets follicles in the anagen phase, which makes up 90% of hair follicles and normally lasts for 2-7 years. The condition has been linked to vitiligo, thyroid disease, and atopy (allergic rhinitis, asthma, and atopic dermatitis). AA has also been linked to autoimmune disorders, such as lupus erythematosus, psoriasis, rheumatoid arthritis, celiac disease, and type 1 diabetes mellitus. In addition, Dr. Hordinsky said, genetic research has linked alopecia to the latter three conditions.

It’s important to ask how much the hair loss bothers the patient, regardless of his or her age, Dr. Hordinsky said. “You need to figure out what’s making them all anxious and depressed. Is it shedding hair every single day?” she said. “Figure out what bothers that patient and go after that issue after you’ve answered his or her questions.”

In some cases, she said, “patients who’ve had extensive disease for a very long time are really coming in for a social visit to talk about what’s new, what’s emerging, whether they’d be interested in participating in a clinical trial.”

Dr. Hordinsky provided this list of questions to help in evaluating these patients:

• Is scalp hair loss the biggest problem? Eyebrow loss? Eyelash loss? Nail issues? All of these?
• How bothersome is it to experience hair shedding every day? Does this make the patient depressed and anxious?

Dr. Hordinsky added that asking patients about the following topics can also be appropriate:

• Hair care habits. Is the patient no longer shampooing because of hair loss?
• Medications.
• Symptoms, such as pain, itch, and burning.
• Body hair. Does the patient feel there’s too little or too much?
• Nail abnormalities.
• Menstrual cycle and pregnancies.
• Diet and supplements.
• Family history. Patients – who are often very curious about the disease – often mention relatives when they hear about a genetic role in AA, she said. They’ll say “My aunt has rheumatoid arthritis” or “My cousin has autoimmune disease.”
• Excess androgen levels and autoimmune/endocrine diseases.

The patient examination should include documentation of the presence of vellus hair or indeterminate and terminal fibers; the presence or absence of scale, erythema, folliculitis, and atrophy; eyebrow, eyelash, or body hair loss; and nail damage, such as pitting.

Photography and/or the SALT score may be used for documentation, she said. The SALT score is the only standardized method of assessment for AA, she said. The score is produced by a calculation that takes into account the amount of hair loss in the quadrants (right, left, front, back) of the scalp (J Am Acad Dermatol. 2018 Sep;79[3]:470-478.e3).

Laboratory tests are important, but there is no standard list, Dr. Hordinsky said. They are selected based on history and physical examination and can include thyroid function studies; heme and iron profiles, including serum ferritin and hemoglobin; and measurements of vitamin D, thiamine, zinc, and total protein. “We check nutrition labs particularly if a patient has been taking a lot of supplements, and we’re not sure where we’re at,” Dr. Hordinsky said.

Dermatologists may also order tests to measure non-cycle dependent hormones such as dehydroepiandrosterone sulfate (DHEAS), total/free testosterone, and antinuclear antibodies and other autoantibodies.

SDEF and this news organization are owned by the same parent company.

Dr. Hordinsky disclosed consulting work with Procter & Gamble, Concert, and Cassiopea and grant/research support from Aclaris, National Alopecia Areata Foundation, Allergan.

Case Report

Four-year-old identical triplet girls with numerous asymptomatic scattered papules on the chest of 4 months’ duration were referred to a dermatologist by their pediatrician for molluscum contagiosum. The patients’ father reported that there was no history of trauma, irritation, or manipulation to the affected area. Their medical history was notable for prematurity at 32 weeks’ gestation and congenital dermal melanocytosis. Family history was notable for their father having acne and similar papules on the chest during adolescence that resolved with isotretinoin therapy.

On physical examination there were multiple smooth, hyperpigmented to erythematous, comedonal, 1- to 2-mm papules dispersed on the anterior central chest of all 3 patients (Figure 1). Clinically, these lesions were fairly indistinguishable from other common dermatologic conditions such as acne or milia. Dermoscopic examination revealed homogenous yellow-white areas surrounded by light brown to erythematous halos (Figure 2). Histopathologic examination was not performed given the benign clinical diagnosis and avoidance of biopsy in pediatric populations. Based on dermoscopic features and history, a diagnosis of eruptive vellus hair cysts (EVHCs) in identical triplets was made.

Comment

Pathogenesis
Eruptive vellus hair cysts, first introduced by Esterly et al¹ in 1977, are uncommon benign lesions presumed to be caused by an abnormal development of the infundibular portion of the hair follicle.² They are usually 1- to 3-mm, reddish brown, monomorphous papules overlapping with pilosebaceous and apocrine units.³ Although the lesions typically are located on the chest and extremities, they may occur on the face, abdomen, axillae, buttocks, or genital area.^1,3 The inheritance of EVHCs is unclear. The majority of reported cases are sporadic; however, the literature mentions 19 families affected by autosomal-dominant EVHCs based on phylogeny.³ In 2015, EVHCs were reported in identical twins, further supporting the case for a genetic mutation.⁴ We augment this autosomal-dominant inheritance pattern by presenting a case of identical triplets with EVHCs. The patients’ father reported similar lesions in childhood, further underscoring a genetic basis.

The pathogenesis of EVHC is uncertain, with 2 main theories. Some propose retention of vellus hair and keratin in a cavity formed by an abnormal vellus hair follicle causing infundibular occlusion. Others consider the growth of benign follicular hamartomas that differentiate to become vellus hairs.¹

Clinical Presentation
The sporadic form of EVHCs is noted to be more common and clinically presents later, with an average age at onset of 16 years and an average age at diagnosis of 24 years.³ The sporadic form occurs without trauma or manipulation as a precursor. Less commonly, lesions present at birth or in early infancy and may show an autosomal-dominant inheritance pattern with a similar distribution across relatives.³

Other variants of EVHCs have been described. Late-onset EVHC usually occurs at 35 years or older (average age, 57 years), with a female to male predominance of 2.5 to 1.³ This late onset may be attributed to proliferation of ductal follicular keratinocytes or loss of perifollicular elastic fibers exacerbated by exogenous factors such as manipulation, UV rays, or trauma.⁵

For unilesional EVHC, the average age at diagnosis is 27 years.³ Some of these lesions may be pedunculated and greater than 8 mm. There is a female to male predominance of 2 to 1. Eruptive vellus hair cysts with steatocystoma multiplex can be seen with an average age at onset of 19 years and a female to male predominance of 0.2 to 1. There may be a family history of this subset, as reported in 3 patients with this pattern.³

Diagnosis
The recommended workup for EVHCs varies by patient and age. Eruptive vellus hair cysts present an opportunity to utilize noninvasive diagnostic procedures, especially for the pediatric population, to avoid scarring and pain from manipulation or biopsies. Although many practitioners may comfortably diagnose EVHCs clinically, 6 cases were misdiagnosed as steatocystoma multiplex, keratosis pilaris, or milia prior to histopathology revealing vellus hair cysts.⁶

Dermoscopy presents as a useful diagnostic aid. Eruptive vellus hair cysts exhibit light yellow homogenous circular structures with a maroon or erythematous halo.^2,7 A central gray-blue color point may be seen due to melanin in the pigmented hair shaft.⁷ A dermoscopy review of EVHCs reported radiating capillaries.² Occasionally, nonfollicular homogenous blue pigmentation may be seen due to a connection to atrophic hair follicles in the mid dermis and no normal hair follicle around the cysts.⁸ In comparison, dermoscopic characteristics of molluscum contagiosum demonstrated a polylobular, white-yellow, amorphous structure at the center with a hardened central umbilicated core and a crown of hairpin vessels at the periphery. Additionally, comedonal acne, commonly mistaken for EVHCs, reveals a brown-yellow hard central plug with sparse inflammation under dermoscopy.² Thus, differentiation of these entities with dermoscopy should be highly prioritized to better aid in the diagnosis of pediatric dermatologic conditions using painless noninvasive techniques.

Treatment
The main indication for treatment of EVHCs is cosmetic concern. Twenty-five percent of EVHCs spontaneously resolve with transepidermal hair elimination or a granulomatous reaction.^4,5 A case report of 4 siblings with congenital EVHCs also described a mother with similar lesions that resolved spontaneously in early adulthood,³ as our patients’ father also noted. Treatment modalities including topical keratolytic agents such as urea 10%, retinoic acid 0.05%, tazarotene cream 0.1%, and lactic acid 12%; incision and drainage; CO₂ laser; or erbium-doped YAG laser ablation have been tried with minimal improvement.⁹ Of note, tazarotene cream 0.1% has demonstrated better results than both erbium-doped YAG laser and drainage and incision of EVHCs.⁴ Additionally, another report evidenced partial improvement with calcipotriene within 2 months with some lesions completely resolved and others flattened, which may be attributed to the antiproliferative and prodifferentiating effects on the ductal follicular keratinocytes by calcipotriene.⁵ Lastly, an additional study indicated that isotretinoin and vitamin A derivatives were ineffective for clearing EVHCs.¹⁰

Conclusion

We presented 3 identical triplets with the classic pediatric onset and dermoscopic findings of EVHCs on the trunk. Although the definitive diagnosis of EVHCs relies on histopathology, we argue that their unique dermoscopic findings combined with a thorough clinical examination is sufficient to recognize this benign condition and avoid painful procedures in the pediatric population.

Case Report

Four-year-old identical triplet girls with numerous asymptomatic scattered papules on the chest of 4 months’ duration were referred to a dermatologist by their pediatrician for molluscum contagiosum. The patients’ father reported that there was no history of trauma, irritation, or manipulation to the affected area. Their medical history was notable for prematurity at 32 weeks’ gestation and congenital dermal melanocytosis. Family history was notable for their father having acne and similar papules on the chest during adolescence that resolved with isotretinoin therapy.

On physical examination there were multiple smooth, hyperpigmented to erythematous, comedonal, 1- to 2-mm papules dispersed on the anterior central chest of all 3 patients (Figure 1). Clinically, these lesions were fairly indistinguishable from other common dermatologic conditions such as acne or milia. Dermoscopic examination revealed homogenous yellow-white areas surrounded by light brown to erythematous halos (Figure 2). Histopathologic examination was not performed given the benign clinical diagnosis and avoidance of biopsy in pediatric populations. Based on dermoscopic features and history, a diagnosis of eruptive vellus hair cysts (EVHCs) in identical triplets was made.

Comment

Pathogenesis
Eruptive vellus hair cysts, first introduced by Esterly et al¹ in 1977, are uncommon benign lesions presumed to be caused by an abnormal development of the infundibular portion of the hair follicle.² They are usually 1- to 3-mm, reddish brown, monomorphous papules overlapping with pilosebaceous and apocrine units.³ Although the lesions typically are located on the chest and extremities, they may occur on the face, abdomen, axillae, buttocks, or genital area.^1,3 The inheritance of EVHCs is unclear. The majority of reported cases are sporadic; however, the literature mentions 19 families affected by autosomal-dominant EVHCs based on phylogeny.³ In 2015, EVHCs were reported in identical twins, further supporting the case for a genetic mutation.⁴ We augment this autosomal-dominant inheritance pattern by presenting a case of identical triplets with EVHCs. The patients’ father reported similar lesions in childhood, further underscoring a genetic basis.

The pathogenesis of EVHC is uncertain, with 2 main theories. Some propose retention of vellus hair and keratin in a cavity formed by an abnormal vellus hair follicle causing infundibular occlusion. Others consider the growth of benign follicular hamartomas that differentiate to become vellus hairs.¹

Clinical Presentation
The sporadic form of EVHCs is noted to be more common and clinically presents later, with an average age at onset of 16 years and an average age at diagnosis of 24 years.³ The sporadic form occurs without trauma or manipulation as a precursor. Less commonly, lesions present at birth or in early infancy and may show an autosomal-dominant inheritance pattern with a similar distribution across relatives.³

Other variants of EVHCs have been described. Late-onset EVHC usually occurs at 35 years or older (average age, 57 years), with a female to male predominance of 2.5 to 1.³ This late onset may be attributed to proliferation of ductal follicular keratinocytes or loss of perifollicular elastic fibers exacerbated by exogenous factors such as manipulation, UV rays, or trauma.⁵

For unilesional EVHC, the average age at diagnosis is 27 years.³ Some of these lesions may be pedunculated and greater than 8 mm. There is a female to male predominance of 2 to 1. Eruptive vellus hair cysts with steatocystoma multiplex can be seen with an average age at onset of 19 years and a female to male predominance of 0.2 to 1. There may be a family history of this subset, as reported in 3 patients with this pattern.³

Diagnosis
The recommended workup for EVHCs varies by patient and age. Eruptive vellus hair cysts present an opportunity to utilize noninvasive diagnostic procedures, especially for the pediatric population, to avoid scarring and pain from manipulation or biopsies. Although many practitioners may comfortably diagnose EVHCs clinically, 6 cases were misdiagnosed as steatocystoma multiplex, keratosis pilaris, or milia prior to histopathology revealing vellus hair cysts.⁶

Dermoscopy presents as a useful diagnostic aid. Eruptive vellus hair cysts exhibit light yellow homogenous circular structures with a maroon or erythematous halo.^2,7 A central gray-blue color point may be seen due to melanin in the pigmented hair shaft.⁷ A dermoscopy review of EVHCs reported radiating capillaries.² Occasionally, nonfollicular homogenous blue pigmentation may be seen due to a connection to atrophic hair follicles in the mid dermis and no normal hair follicle around the cysts.⁸ In comparison, dermoscopic characteristics of molluscum contagiosum demonstrated a polylobular, white-yellow, amorphous structure at the center with a hardened central umbilicated core and a crown of hairpin vessels at the periphery. Additionally, comedonal acne, commonly mistaken for EVHCs, reveals a brown-yellow hard central plug with sparse inflammation under dermoscopy.² Thus, differentiation of these entities with dermoscopy should be highly prioritized to better aid in the diagnosis of pediatric dermatologic conditions using painless noninvasive techniques.

Treatment
The main indication for treatment of EVHCs is cosmetic concern. Twenty-five percent of EVHCs spontaneously resolve with transepidermal hair elimination or a granulomatous reaction.^4,5 A case report of 4 siblings with congenital EVHCs also described a mother with similar lesions that resolved spontaneously in early adulthood,³ as our patients’ father also noted. Treatment modalities including topical keratolytic agents such as urea 10%, retinoic acid 0.05%, tazarotene cream 0.1%, and lactic acid 12%; incision and drainage; CO₂ laser; or erbium-doped YAG laser ablation have been tried with minimal improvement.⁹ Of note, tazarotene cream 0.1% has demonstrated better results than both erbium-doped YAG laser and drainage and incision of EVHCs.⁴ Additionally, another report evidenced partial improvement with calcipotriene within 2 months with some lesions completely resolved and others flattened, which may be attributed to the antiproliferative and prodifferentiating effects on the ductal follicular keratinocytes by calcipotriene.⁵ Lastly, an additional study indicated that isotretinoin and vitamin A derivatives were ineffective for clearing EVHCs.¹⁰

Conclusion

We presented 3 identical triplets with the classic pediatric onset and dermoscopic findings of EVHCs on the trunk. Although the definitive diagnosis of EVHCs relies on histopathology, we argue that their unique dermoscopic findings combined with a thorough clinical examination is sufficient to recognize this benign condition and avoid painful procedures in the pediatric population.

Case Report

Four-year-old identical triplet girls with numerous asymptomatic scattered papules on the chest of 4 months’ duration were referred to a dermatologist by their pediatrician for molluscum contagiosum. The patients’ father reported that there was no history of trauma, irritation, or manipulation to the affected area. Their medical history was notable for prematurity at 32 weeks’ gestation and congenital dermal melanocytosis. Family history was notable for their father having acne and similar papules on the chest during adolescence that resolved with isotretinoin therapy.

On physical examination there were multiple smooth, hyperpigmented to erythematous, comedonal, 1- to 2-mm papules dispersed on the anterior central chest of all 3 patients (Figure 1). Clinically, these lesions were fairly indistinguishable from other common dermatologic conditions such as acne or milia. Dermoscopic examination revealed homogenous yellow-white areas surrounded by light brown to erythematous halos (Figure 2). Histopathologic examination was not performed given the benign clinical diagnosis and avoidance of biopsy in pediatric populations. Based on dermoscopic features and history, a diagnosis of eruptive vellus hair cysts (EVHCs) in identical triplets was made.

Comment

Pathogenesis
Eruptive vellus hair cysts, first introduced by Esterly et al¹ in 1977, are uncommon benign lesions presumed to be caused by an abnormal development of the infundibular portion of the hair follicle.² They are usually 1- to 3-mm, reddish brown, monomorphous papules overlapping with pilosebaceous and apocrine units.³ Although the lesions typically are located on the chest and extremities, they may occur on the face, abdomen, axillae, buttocks, or genital area.^1,3 The inheritance of EVHCs is unclear. The majority of reported cases are sporadic; however, the literature mentions 19 families affected by autosomal-dominant EVHCs based on phylogeny.³ In 2015, EVHCs were reported in identical twins, further supporting the case for a genetic mutation.⁴ We augment this autosomal-dominant inheritance pattern by presenting a case of identical triplets with EVHCs. The patients’ father reported similar lesions in childhood, further underscoring a genetic basis.

The pathogenesis of EVHC is uncertain, with 2 main theories. Some propose retention of vellus hair and keratin in a cavity formed by an abnormal vellus hair follicle causing infundibular occlusion. Others consider the growth of benign follicular hamartomas that differentiate to become vellus hairs.¹

Clinical Presentation
The sporadic form of EVHCs is noted to be more common and clinically presents later, with an average age at onset of 16 years and an average age at diagnosis of 24 years.³ The sporadic form occurs without trauma or manipulation as a precursor. Less commonly, lesions present at birth or in early infancy and may show an autosomal-dominant inheritance pattern with a similar distribution across relatives.³

Other variants of EVHCs have been described. Late-onset EVHC usually occurs at 35 years or older (average age, 57 years), with a female to male predominance of 2.5 to 1.³ This late onset may be attributed to proliferation of ductal follicular keratinocytes or loss of perifollicular elastic fibers exacerbated by exogenous factors such as manipulation, UV rays, or trauma.⁵

For unilesional EVHC, the average age at diagnosis is 27 years.³ Some of these lesions may be pedunculated and greater than 8 mm. There is a female to male predominance of 2 to 1. Eruptive vellus hair cysts with steatocystoma multiplex can be seen with an average age at onset of 19 years and a female to male predominance of 0.2 to 1. There may be a family history of this subset, as reported in 3 patients with this pattern.³

Diagnosis
The recommended workup for EVHCs varies by patient and age. Eruptive vellus hair cysts present an opportunity to utilize noninvasive diagnostic procedures, especially for the pediatric population, to avoid scarring and pain from manipulation or biopsies. Although many practitioners may comfortably diagnose EVHCs clinically, 6 cases were misdiagnosed as steatocystoma multiplex, keratosis pilaris, or milia prior to histopathology revealing vellus hair cysts.⁶

Dermoscopy presents as a useful diagnostic aid. Eruptive vellus hair cysts exhibit light yellow homogenous circular structures with a maroon or erythematous halo.^2,7 A central gray-blue color point may be seen due to melanin in the pigmented hair shaft.⁷ A dermoscopy review of EVHCs reported radiating capillaries.² Occasionally, nonfollicular homogenous blue pigmentation may be seen due to a connection to atrophic hair follicles in the mid dermis and no normal hair follicle around the cysts.⁸ In comparison, dermoscopic characteristics of molluscum contagiosum demonstrated a polylobular, white-yellow, amorphous structure at the center with a hardened central umbilicated core and a crown of hairpin vessels at the periphery. Additionally, comedonal acne, commonly mistaken for EVHCs, reveals a brown-yellow hard central plug with sparse inflammation under dermoscopy.² Thus, differentiation of these entities with dermoscopy should be highly prioritized to better aid in the diagnosis of pediatric dermatologic conditions using painless noninvasive techniques.

Treatment
The main indication for treatment of EVHCs is cosmetic concern. Twenty-five percent of EVHCs spontaneously resolve with transepidermal hair elimination or a granulomatous reaction.^4,5 A case report of 4 siblings with congenital EVHCs also described a mother with similar lesions that resolved spontaneously in early adulthood,³ as our patients’ father also noted. Treatment modalities including topical keratolytic agents such as urea 10%, retinoic acid 0.05%, tazarotene cream 0.1%, and lactic acid 12%; incision and drainage; CO₂ laser; or erbium-doped YAG laser ablation have been tried with minimal improvement.⁹ Of note, tazarotene cream 0.1% has demonstrated better results than both erbium-doped YAG laser and drainage and incision of EVHCs.⁴ Additionally, another report evidenced partial improvement with calcipotriene within 2 months with some lesions completely resolved and others flattened, which may be attributed to the antiproliferative and prodifferentiating effects on the ductal follicular keratinocytes by calcipotriene.⁵ Lastly, an additional study indicated that isotretinoin and vitamin A derivatives were ineffective for clearing EVHCs.¹⁰

Conclusion

We presented 3 identical triplets with the classic pediatric onset and dermoscopic findings of EVHCs on the trunk. Although the definitive diagnosis of EVHCs relies on histopathology, we argue that their unique dermoscopic findings combined with a thorough clinical examination is sufficient to recognize this benign condition and avoid painful procedures in the pediatric population.

Frontal fibrosing alopecia (FFA) has been reported in association with lichen planus pigmentosus (LPP) and facial papules.^1-3 Lichen planus pigmentosus is a variant of lichen planus that causes hyperpigmentation of the face, neck, and/or intertriginous areas that may be useful as a clinical indicator in the development of FFA.¹ Facial papules in association with FFA are secondary to fibrosed vellus hairs.^2,3 Currently, reports of concomitant FFA, LPP, and facial papules in women with skin of color are limited in the literature. This case series includes 5 women of color (Hispanic and black) who presented to our clinic with FFA and various cutaneous associations. A review of the current literature on cutaneous associations of FFA also is provided.

Case Reports

Patient 1
A 50-year-old Hispanic woman who was previously presumed to have melasma by an outside physician presented with pruritus of the scalp and eyebrows of 1 month’s duration. Physical examination revealed decreased frontal scalp hair density with perifollicular erythema and scale with thinning of the lateral eyebrows. Hyperpigmented coalesced macules (Figure 1A) and erythematous perifollicular papules were noted along the temples and on the perioral skin. Depressed forehead and temporal veins also were noted (Figure 1B). A biopsy of the scalp demonstrated perifollicular and perivascular lymphocytic inflammation and fibrosed hair follicles, and a biopsy of the perioral skin demonstrated perivascular lymphocytic inflammation with melanophages in the papillary dermis. A diagnosis of FFA with LPP was established with these biopsies.

Patient 2
A 61-year-old black woman presented with asymptomatic hair loss along the frontal hairline for an unknown duration. On physical examination the frontal scalp and lateral eyebrows demonstrated decreased hair density with loss of follicular ostia. Fine, flesh-colored, monomorphic papules were scattered along the forehead and temples, and ill-defined brown pigmentation was present along the forehead, temples, and cheeks. Biopsy of the frontal scalp demonstrated patchy lichenoid inflammation with decreased number of follicles with replacement by follicular scars, confirming the diagnosis of FFA.

Patient 3
A 47-year-old Hispanic woman presented with hair loss of the frontal scalp and bilateral eyebrows with associated burning of 2 years’ duration. Physical examination demonstrated recession of the frontotemporal hairline with scattered lone hairs and thinning of the eyebrows. Innumerable flesh-colored papules were present on the forehead and temples (Figure 2A). Glabellar and eyebrow erythema was noted (Figure 2B). Biopsy of the frontal scalp demonstrated decreased terminal anagen hair follicles with perifollicular lymphoid infiltrate and fibrosis, consistent with a diagnosis of FFA. The patient was started on oral hydroxychloroquine 400 mg once daily, and 3 months later hyperpigmentation of the forehead and perioral skin was noted. The patient reported that she had facial hyperpigmentation prior to starting hydroxychloroquine and declined a biopsy.

Patient 4
A 40-year-old black woman presented with brown pruritic macles of the face, neck, arms, and forearms of 4 years’ duration. She also reported hair loss on the frontal and occipital scalp, eyebrows, and arms. On physical examination, ill-defined brown macules and patches were noted on the neck (Figure 3), face, arms, and forearms. Decreased hair density was noted on the frontal and occipital scalp with follicular dropout and perifollicular hyperpigmentation. Biopsy of the scalp demonstrated perivascular lymphocytic inflammation with sparse anagen follicles and fibrous tracts, and biopsy of the neck revealed superficial perivascular inflammation with numerous melanophages in the upper dermis; these histopathologic findings were consistent with FFA and LPP, respectively.

Patient 5
A 46-year-old black woman with history of hair loss presented with hyperpigmentation of the face and neck of 2 years’ duration. On physical examination decreased hair density of the frontal and vertex scalp and lateral eyebrows was noted. Flesh-colored papules were noted on the forehead and cheeks, and confluent dark brown patches were present on the temples and neck. Three punch biopsies were performed. Biopsy of the scalp revealed lymphocytic inflammation with surrounding fibroplasia with overlapping features of FFA and central centrifugal cicatricial alopecia (Figure 4). Biopsy of the neck revealed vacuolar interface dermatitis. Additionally, biopsy of a facial papule revealed lichenoid inflammation involving a vellus hair follicle. Clinical and histopathological correlation confirmed the diagnosis of FFA with LPP and facial papules.

Comment

Current understanding of FFA as a progressive, lymphocytic, scarring alopecia has expanded in recent years. Clinical observation suggests that the incidence of FFA is increasing⁴; however more epidemiologic data are needed. Frontal fibrosing alopecia presents clinically with symmetrical frontotemporal hair loss with lone hairs. Trichoscopy reveals perifollicular hyperkeratosis, perifollicular erythema, and follicular plugging in 72%, 66%, and 44% of cases, respectively.⁵ In one study (N=242), patients were classified into 3 clinical patterns of FFA: pattern I (linear) showed bandlike loss of frontal hair with normal density directly behind the hairline; pattern II (diffuse) showed loss of density behind the frontal hairline; and pattern III (double line) showed a pseudo–“fringe sign” appearance. The majority of patients were classified as either pattern I or II, with pattern II predicting a poorer prognosis.⁶

rontal fibrosing alopecia is increasingly recognized in men, with prevalence as high as 5%.¹ Facial hair involvement, particularly of the upper lip and sideburns, is an important consideration in men.⁷ Most studies suggest that 80% to 90% of affected women are postmenopausal,⁸ though a case series presented by Dlova¹ identified 27% of affected women as postmenopausal. The coexistence of premature menopause and hysterectomy in FFA patients suggests a hormonal contribution, but this association is still poorly understood.⁸ Epidemiologic data on ethnicity in FFA are sparse but suggest that white individuals are more likely to be affected. Frontal fibrosing alopecia also may be misdiagnosed as traction alopecia in Hispanic and black patients.⁸

It is prudent for physicians to assess for and recognize clinical clues to severe forms of FFA. A 2014 multicenter review of 355 patients identified 3 clinical entities that predicted more severe forms of FFA: eyelash loss (madarosis), loss of body hair, and facial papules.⁸ Madarosis occurs due to perifollicular inflammation and fibrosis of eyelash hair follicles. Similarly, perifollicular inflammation of body hair was present in 24% of patients (N=86), most commonly of the axillary and pubic hair. Facial papules form due to facial vellus hair inflammation and fibrosis and were identified in 14% of patients (N=49).⁸ These clinical findings may allow providers to predict more extensive clinical involvement of FFA.

Frontal fibrosing alopecia and LPP occur concomitantly in up 54% of patients, more commonly in darker-skinned patients.^1,9,10 Lichen planus pigmentosus frequently occurs on the face and neck, most commonly in a diffuse pattern, though reticulated and macular patterns also have been identified.¹¹ In some patients, LPP precedes the development of FFA and may be useful as a herald sign¹; therefore, it is important for dermatologists to evaluate for signs of FFA when evaluating those with LPP. Thorough evaluation in patients with skin of color also is important because FFA may be misdiagnosed as traction alopecia.

Additional cutaneous associations of FFA include eyebrow loss, glabellar red dots, and prominent frontal veins. Eyebrow loss occurs secondary to fibrosis of eyebrow hair follicles and has been found in 40% to 80% of patients with FFA; it is thought to be associated with milder forms of FFA.⁸ Glabellar red dots correlate with histopathologic lymphocytic inflammation of vellus hair follicles.¹² Additionally, frontal vein prominence has been described in FFA and is thought to be secondary to atrophy in this scarring process, perhaps worsened by local steroid treatments.¹³ Mucocutaneous lichen planus, rosacea, thyroid disease, vitiligo, and other autoimmune disorders also have been reported in patients with FFA.¹⁴

Conclusion

Concomitant FFA, LPP, and facial papules have been rarely reported and exemplify the spectrum of cutaneous associations with FFA, particularly in individuals with skin of color. Clinical variants and associations of FFA are broad, including predictors of poorer prognosis such as eyelash loss and vellus hair involvement seen as facial papules. Lichen planus pigmentosus is well described in association with FFA and may serve as a herald sign that frontal hair loss should not be mistaken for traction alopecia in early stages. Eyebrow loss is thought to represent milder disease. It is important for dermatologists to be aware of these findings to understand the breadth of this disease and for appropriate evaluation and management of patients with FFA.

Frontal fibrosing alopecia (FFA) has been reported in association with lichen planus pigmentosus (LPP) and facial papules.^1-3 Lichen planus pigmentosus is a variant of lichen planus that causes hyperpigmentation of the face, neck, and/or intertriginous areas that may be useful as a clinical indicator in the development of FFA.¹ Facial papules in association with FFA are secondary to fibrosed vellus hairs.^2,3 Currently, reports of concomitant FFA, LPP, and facial papules in women with skin of color are limited in the literature. This case series includes 5 women of color (Hispanic and black) who presented to our clinic with FFA and various cutaneous associations. A review of the current literature on cutaneous associations of FFA also is provided.

Case Reports

Patient 1
A 50-year-old Hispanic woman who was previously presumed to have melasma by an outside physician presented with pruritus of the scalp and eyebrows of 1 month’s duration. Physical examination revealed decreased frontal scalp hair density with perifollicular erythema and scale with thinning of the lateral eyebrows. Hyperpigmented coalesced macules (Figure 1A) and erythematous perifollicular papules were noted along the temples and on the perioral skin. Depressed forehead and temporal veins also were noted (Figure 1B). A biopsy of the scalp demonstrated perifollicular and perivascular lymphocytic inflammation and fibrosed hair follicles, and a biopsy of the perioral skin demonstrated perivascular lymphocytic inflammation with melanophages in the papillary dermis. A diagnosis of FFA with LPP was established with these biopsies.

Patient 2
A 61-year-old black woman presented with asymptomatic hair loss along the frontal hairline for an unknown duration. On physical examination the frontal scalp and lateral eyebrows demonstrated decreased hair density with loss of follicular ostia. Fine, flesh-colored, monomorphic papules were scattered along the forehead and temples, and ill-defined brown pigmentation was present along the forehead, temples, and cheeks. Biopsy of the frontal scalp demonstrated patchy lichenoid inflammation with decreased number of follicles with replacement by follicular scars, confirming the diagnosis of FFA.

Patient 3
A 47-year-old Hispanic woman presented with hair loss of the frontal scalp and bilateral eyebrows with associated burning of 2 years’ duration. Physical examination demonstrated recession of the frontotemporal hairline with scattered lone hairs and thinning of the eyebrows. Innumerable flesh-colored papules were present on the forehead and temples (Figure 2A). Glabellar and eyebrow erythema was noted (Figure 2B). Biopsy of the frontal scalp demonstrated decreased terminal anagen hair follicles with perifollicular lymphoid infiltrate and fibrosis, consistent with a diagnosis of FFA. The patient was started on oral hydroxychloroquine 400 mg once daily, and 3 months later hyperpigmentation of the forehead and perioral skin was noted. The patient reported that she had facial hyperpigmentation prior to starting hydroxychloroquine and declined a biopsy.

Patient 4
A 40-year-old black woman presented with brown pruritic macles of the face, neck, arms, and forearms of 4 years’ duration. She also reported hair loss on the frontal and occipital scalp, eyebrows, and arms. On physical examination, ill-defined brown macules and patches were noted on the neck (Figure 3), face, arms, and forearms. Decreased hair density was noted on the frontal and occipital scalp with follicular dropout and perifollicular hyperpigmentation. Biopsy of the scalp demonstrated perivascular lymphocytic inflammation with sparse anagen follicles and fibrous tracts, and biopsy of the neck revealed superficial perivascular inflammation with numerous melanophages in the upper dermis; these histopathologic findings were consistent with FFA and LPP, respectively.

Patient 5
A 46-year-old black woman with history of hair loss presented with hyperpigmentation of the face and neck of 2 years’ duration. On physical examination decreased hair density of the frontal and vertex scalp and lateral eyebrows was noted. Flesh-colored papules were noted on the forehead and cheeks, and confluent dark brown patches were present on the temples and neck. Three punch biopsies were performed. Biopsy of the scalp revealed lymphocytic inflammation with surrounding fibroplasia with overlapping features of FFA and central centrifugal cicatricial alopecia (Figure 4). Biopsy of the neck revealed vacuolar interface dermatitis. Additionally, biopsy of a facial papule revealed lichenoid inflammation involving a vellus hair follicle. Clinical and histopathological correlation confirmed the diagnosis of FFA with LPP and facial papules.

Comment

Current understanding of FFA as a progressive, lymphocytic, scarring alopecia has expanded in recent years. Clinical observation suggests that the incidence of FFA is increasing⁴; however more epidemiologic data are needed. Frontal fibrosing alopecia presents clinically with symmetrical frontotemporal hair loss with lone hairs. Trichoscopy reveals perifollicular hyperkeratosis, perifollicular erythema, and follicular plugging in 72%, 66%, and 44% of cases, respectively.⁵ In one study (N=242), patients were classified into 3 clinical patterns of FFA: pattern I (linear) showed bandlike loss of frontal hair with normal density directly behind the hairline; pattern II (diffuse) showed loss of density behind the frontal hairline; and pattern III (double line) showed a pseudo–“fringe sign” appearance. The majority of patients were classified as either pattern I or II, with pattern II predicting a poorer prognosis.⁶

rontal fibrosing alopecia is increasingly recognized in men, with prevalence as high as 5%.¹ Facial hair involvement, particularly of the upper lip and sideburns, is an important consideration in men.⁷ Most studies suggest that 80% to 90% of affected women are postmenopausal,⁸ though a case series presented by Dlova¹ identified 27% of affected women as postmenopausal. The coexistence of premature menopause and hysterectomy in FFA patients suggests a hormonal contribution, but this association is still poorly understood.⁸ Epidemiologic data on ethnicity in FFA are sparse but suggest that white individuals are more likely to be affected. Frontal fibrosing alopecia also may be misdiagnosed as traction alopecia in Hispanic and black patients.⁸

It is prudent for physicians to assess for and recognize clinical clues to severe forms of FFA. A 2014 multicenter review of 355 patients identified 3 clinical entities that predicted more severe forms of FFA: eyelash loss (madarosis), loss of body hair, and facial papules.⁸ Madarosis occurs due to perifollicular inflammation and fibrosis of eyelash hair follicles. Similarly, perifollicular inflammation of body hair was present in 24% of patients (N=86), most commonly of the axillary and pubic hair. Facial papules form due to facial vellus hair inflammation and fibrosis and were identified in 14% of patients (N=49).⁸ These clinical findings may allow providers to predict more extensive clinical involvement of FFA.

Frontal fibrosing alopecia and LPP occur concomitantly in up 54% of patients, more commonly in darker-skinned patients.^1,9,10 Lichen planus pigmentosus frequently occurs on the face and neck, most commonly in a diffuse pattern, though reticulated and macular patterns also have been identified.¹¹ In some patients, LPP precedes the development of FFA and may be useful as a herald sign¹; therefore, it is important for dermatologists to evaluate for signs of FFA when evaluating those with LPP. Thorough evaluation in patients with skin of color also is important because FFA may be misdiagnosed as traction alopecia.

Additional cutaneous associations of FFA include eyebrow loss, glabellar red dots, and prominent frontal veins. Eyebrow loss occurs secondary to fibrosis of eyebrow hair follicles and has been found in 40% to 80% of patients with FFA; it is thought to be associated with milder forms of FFA.⁸ Glabellar red dots correlate with histopathologic lymphocytic inflammation of vellus hair follicles.¹² Additionally, frontal vein prominence has been described in FFA and is thought to be secondary to atrophy in this scarring process, perhaps worsened by local steroid treatments.¹³ Mucocutaneous lichen planus, rosacea, thyroid disease, vitiligo, and other autoimmune disorders also have been reported in patients with FFA.¹⁴

Conclusion

Concomitant FFA, LPP, and facial papules have been rarely reported and exemplify the spectrum of cutaneous associations with FFA, particularly in individuals with skin of color. Clinical variants and associations of FFA are broad, including predictors of poorer prognosis such as eyelash loss and vellus hair involvement seen as facial papules. Lichen planus pigmentosus is well described in association with FFA and may serve as a herald sign that frontal hair loss should not be mistaken for traction alopecia in early stages. Eyebrow loss is thought to represent milder disease. It is important for dermatologists to be aware of these findings to understand the breadth of this disease and for appropriate evaluation and management of patients with FFA.

Frontal fibrosing alopecia (FFA) has been reported in association with lichen planus pigmentosus (LPP) and facial papules.^1-3 Lichen planus pigmentosus is a variant of lichen planus that causes hyperpigmentation of the face, neck, and/or intertriginous areas that may be useful as a clinical indicator in the development of FFA.¹ Facial papules in association with FFA are secondary to fibrosed vellus hairs.^2,3 Currently, reports of concomitant FFA, LPP, and facial papules in women with skin of color are limited in the literature. This case series includes 5 women of color (Hispanic and black) who presented to our clinic with FFA and various cutaneous associations. A review of the current literature on cutaneous associations of FFA also is provided.

Case Reports

Patient 1
A 50-year-old Hispanic woman who was previously presumed to have melasma by an outside physician presented with pruritus of the scalp and eyebrows of 1 month’s duration. Physical examination revealed decreased frontal scalp hair density with perifollicular erythema and scale with thinning of the lateral eyebrows. Hyperpigmented coalesced macules (Figure 1A) and erythematous perifollicular papules were noted along the temples and on the perioral skin. Depressed forehead and temporal veins also were noted (Figure 1B). A biopsy of the scalp demonstrated perifollicular and perivascular lymphocytic inflammation and fibrosed hair follicles, and a biopsy of the perioral skin demonstrated perivascular lymphocytic inflammation with melanophages in the papillary dermis. A diagnosis of FFA with LPP was established with these biopsies.

Patient 2
A 61-year-old black woman presented with asymptomatic hair loss along the frontal hairline for an unknown duration. On physical examination the frontal scalp and lateral eyebrows demonstrated decreased hair density with loss of follicular ostia. Fine, flesh-colored, monomorphic papules were scattered along the forehead and temples, and ill-defined brown pigmentation was present along the forehead, temples, and cheeks. Biopsy of the frontal scalp demonstrated patchy lichenoid inflammation with decreased number of follicles with replacement by follicular scars, confirming the diagnosis of FFA.

Patient 3
A 47-year-old Hispanic woman presented with hair loss of the frontal scalp and bilateral eyebrows with associated burning of 2 years’ duration. Physical examination demonstrated recession of the frontotemporal hairline with scattered lone hairs and thinning of the eyebrows. Innumerable flesh-colored papules were present on the forehead and temples (Figure 2A). Glabellar and eyebrow erythema was noted (Figure 2B). Biopsy of the frontal scalp demonstrated decreased terminal anagen hair follicles with perifollicular lymphoid infiltrate and fibrosis, consistent with a diagnosis of FFA. The patient was started on oral hydroxychloroquine 400 mg once daily, and 3 months later hyperpigmentation of the forehead and perioral skin was noted. The patient reported that she had facial hyperpigmentation prior to starting hydroxychloroquine and declined a biopsy.

Patient 4
A 40-year-old black woman presented with brown pruritic macles of the face, neck, arms, and forearms of 4 years’ duration. She also reported hair loss on the frontal and occipital scalp, eyebrows, and arms. On physical examination, ill-defined brown macules and patches were noted on the neck (Figure 3), face, arms, and forearms. Decreased hair density was noted on the frontal and occipital scalp with follicular dropout and perifollicular hyperpigmentation. Biopsy of the scalp demonstrated perivascular lymphocytic inflammation with sparse anagen follicles and fibrous tracts, and biopsy of the neck revealed superficial perivascular inflammation with numerous melanophages in the upper dermis; these histopathologic findings were consistent with FFA and LPP, respectively.

Patient 5
A 46-year-old black woman with history of hair loss presented with hyperpigmentation of the face and neck of 2 years’ duration. On physical examination decreased hair density of the frontal and vertex scalp and lateral eyebrows was noted. Flesh-colored papules were noted on the forehead and cheeks, and confluent dark brown patches were present on the temples and neck. Three punch biopsies were performed. Biopsy of the scalp revealed lymphocytic inflammation with surrounding fibroplasia with overlapping features of FFA and central centrifugal cicatricial alopecia (Figure 4). Biopsy of the neck revealed vacuolar interface dermatitis. Additionally, biopsy of a facial papule revealed lichenoid inflammation involving a vellus hair follicle. Clinical and histopathological correlation confirmed the diagnosis of FFA with LPP and facial papules.

Comment

Current understanding of FFA as a progressive, lymphocytic, scarring alopecia has expanded in recent years. Clinical observation suggests that the incidence of FFA is increasing⁴; however more epidemiologic data are needed. Frontal fibrosing alopecia presents clinically with symmetrical frontotemporal hair loss with lone hairs. Trichoscopy reveals perifollicular hyperkeratosis, perifollicular erythema, and follicular plugging in 72%, 66%, and 44% of cases, respectively.⁵ In one study (N=242), patients were classified into 3 clinical patterns of FFA: pattern I (linear) showed bandlike loss of frontal hair with normal density directly behind the hairline; pattern II (diffuse) showed loss of density behind the frontal hairline; and pattern III (double line) showed a pseudo–“fringe sign” appearance. The majority of patients were classified as either pattern I or II, with pattern II predicting a poorer prognosis.⁶

rontal fibrosing alopecia is increasingly recognized in men, with prevalence as high as 5%.¹ Facial hair involvement, particularly of the upper lip and sideburns, is an important consideration in men.⁷ Most studies suggest that 80% to 90% of affected women are postmenopausal,⁸ though a case series presented by Dlova¹ identified 27% of affected women as postmenopausal. The coexistence of premature menopause and hysterectomy in FFA patients suggests a hormonal contribution, but this association is still poorly understood.⁸ Epidemiologic data on ethnicity in FFA are sparse but suggest that white individuals are more likely to be affected. Frontal fibrosing alopecia also may be misdiagnosed as traction alopecia in Hispanic and black patients.⁸

It is prudent for physicians to assess for and recognize clinical clues to severe forms of FFA. A 2014 multicenter review of 355 patients identified 3 clinical entities that predicted more severe forms of FFA: eyelash loss (madarosis), loss of body hair, and facial papules.⁸ Madarosis occurs due to perifollicular inflammation and fibrosis of eyelash hair follicles. Similarly, perifollicular inflammation of body hair was present in 24% of patients (N=86), most commonly of the axillary and pubic hair. Facial papules form due to facial vellus hair inflammation and fibrosis and were identified in 14% of patients (N=49).⁸ These clinical findings may allow providers to predict more extensive clinical involvement of FFA.

Frontal fibrosing alopecia and LPP occur concomitantly in up 54% of patients, more commonly in darker-skinned patients.^1,9,10 Lichen planus pigmentosus frequently occurs on the face and neck, most commonly in a diffuse pattern, though reticulated and macular patterns also have been identified.¹¹ In some patients, LPP precedes the development of FFA and may be useful as a herald sign¹; therefore, it is important for dermatologists to evaluate for signs of FFA when evaluating those with LPP. Thorough evaluation in patients with skin of color also is important because FFA may be misdiagnosed as traction alopecia.

Additional cutaneous associations of FFA include eyebrow loss, glabellar red dots, and prominent frontal veins. Eyebrow loss occurs secondary to fibrosis of eyebrow hair follicles and has been found in 40% to 80% of patients with FFA; it is thought to be associated with milder forms of FFA.⁸ Glabellar red dots correlate with histopathologic lymphocytic inflammation of vellus hair follicles.¹² Additionally, frontal vein prominence has been described in FFA and is thought to be secondary to atrophy in this scarring process, perhaps worsened by local steroid treatments.¹³ Mucocutaneous lichen planus, rosacea, thyroid disease, vitiligo, and other autoimmune disorders also have been reported in patients with FFA.¹⁴

Conclusion

Concomitant FFA, LPP, and facial papules have been rarely reported and exemplify the spectrum of cutaneous associations with FFA, particularly in individuals with skin of color. Clinical variants and associations of FFA are broad, including predictors of poorer prognosis such as eyelash loss and vellus hair involvement seen as facial papules. Lichen planus pigmentosus is well described in association with FFA and may serve as a herald sign that frontal hair loss should not be mistaken for traction alopecia in early stages. Eyebrow loss is thought to represent milder disease. It is important for dermatologists to be aware of these findings to understand the breadth of this disease and for appropriate evaluation and management of patients with FFA.

The Diagnosis: Pediculosis Pubis

Dermoscopy of the pubic hair demonstrated a louse clutching multiple shafts of hairs (Figure) as well as scattered nits, confirming the presence of Phthirus pubis and diagnosis of pediculosis pubis. The key clinical diagnostic feature in this case was severe itching of the pubic area, with visible nits present on pubic hairs. Itching and infestation also can involve other hair-bearing areas such as the chest, legs, and axillae. The patient was treated with permethrin cream 5% applied to the pubic area and chest. Symptoms and infestation were resolved at 1-week follow-up.

Pediculosis pubis is an infestation of pubic hairs by the pubic (crab) louse P pubis, which feeds on host blood. Other body areas covered with dense hair also may be involved; 60% of patients are infested in at least 2 different sites.¹ Pediculosis pubis is most commonly sexually transmitted through direct contact.² Worldwide prevalence has been estimated at approximately 2% of the adult population, and a survey of 817 US college students in 2009 indicated a lifetime prevalence of 1.3%.³ The prevalence is slightly higher in men, highest in men who have sex with men, and rare in individuals with shaved pubic hair.¹ The most common symptom is pruritus of the genital area. Infested patients also may develop asymptomatic bluish gray macules (maculae ceruleae) secondary to hemosiderin deposition from louse bites.⁴

The diagnosis of pediculosis pubis is made by identification of P pubis, either by examination with the naked eye or confirmation with dermoscopy or microscopy. Although the 0.8- to 1.2-mm lice are visible to the naked eye, they can be difficult to see if not filled with blood, and nits on the hairs can be mistaken for white piedra (fungal infection of the hair shafts) or trichomycosis pubis (bacterial infection of the hair shafts).⁴ Scabies and tinea cruris do not present with attachments to the hairs. Scabies may present with papules and burrows and tinea cruris with scaly erythematous plaques. Small numbers of lice and nits may be missed by the naked eye or a traditional magnifying glass.⁵ The use of dermoscopy allows for fast and accurate identification of the characteristic lice and nits, even in these more challenging cases.⁵ Accurate diagnosis is important, as approximately 31% of infested patients have other concurrent sexually transmitted infections that warrant screening.⁶

The Centers for Disease Control and Prevention recommends first-line treatment with permethrin cream (1% or 5%) or pyrethrin with piperonyl butoxide applied to all affected areas and washed off after 10 minutes.⁷ Patients should be reevaluated after 1 week if symptoms persist and re-treated if lice are found on examination.^7,8 Malathion lotion 0.5% (applied and washed off after 8-12 hours) or oral ivermectin (250 µg/kg, repeated after 2 weeks) may be used for alternative therapies or cases of permethrin or pyrethrin resistance.⁷ Ivermectin also is effective for involvement of eyelashes where topical insecticides should not be used.⁹ Sexual partners should be treated to prevent repeat transmission.^7,8 Bedding and clothing can be decontaminated by machine wash on hot cycle or isolating from body contact for 72 hours.⁷ Patients also should be screened for other sexually transmitted infections, including human immunodeficiency virus.⁶

The Diagnosis: Pediculosis Pubis

Dermoscopy of the pubic hair demonstrated a louse clutching multiple shafts of hairs (Figure) as well as scattered nits, confirming the presence of Phthirus pubis and diagnosis of pediculosis pubis. The key clinical diagnostic feature in this case was severe itching of the pubic area, with visible nits present on pubic hairs. Itching and infestation also can involve other hair-bearing areas such as the chest, legs, and axillae. The patient was treated with permethrin cream 5% applied to the pubic area and chest. Symptoms and infestation were resolved at 1-week follow-up.

Pediculosis pubis is an infestation of pubic hairs by the pubic (crab) louse P pubis, which feeds on host blood. Other body areas covered with dense hair also may be involved; 60% of patients are infested in at least 2 different sites.¹ Pediculosis pubis is most commonly sexually transmitted through direct contact.² Worldwide prevalence has been estimated at approximately 2% of the adult population, and a survey of 817 US college students in 2009 indicated a lifetime prevalence of 1.3%.³ The prevalence is slightly higher in men, highest in men who have sex with men, and rare in individuals with shaved pubic hair.¹ The most common symptom is pruritus of the genital area. Infested patients also may develop asymptomatic bluish gray macules (maculae ceruleae) secondary to hemosiderin deposition from louse bites.⁴

The diagnosis of pediculosis pubis is made by identification of P pubis, either by examination with the naked eye or confirmation with dermoscopy or microscopy. Although the 0.8- to 1.2-mm lice are visible to the naked eye, they can be difficult to see if not filled with blood, and nits on the hairs can be mistaken for white piedra (fungal infection of the hair shafts) or trichomycosis pubis (bacterial infection of the hair shafts).⁴ Scabies and tinea cruris do not present with attachments to the hairs. Scabies may present with papules and burrows and tinea cruris with scaly erythematous plaques. Small numbers of lice and nits may be missed by the naked eye or a traditional magnifying glass.⁵ The use of dermoscopy allows for fast and accurate identification of the characteristic lice and nits, even in these more challenging cases.⁵ Accurate diagnosis is important, as approximately 31% of infested patients have other concurrent sexually transmitted infections that warrant screening.⁶

The Centers for Disease Control and Prevention recommends first-line treatment with permethrin cream (1% or 5%) or pyrethrin with piperonyl butoxide applied to all affected areas and washed off after 10 minutes.⁷ Patients should be reevaluated after 1 week if symptoms persist and re-treated if lice are found on examination.^7,8 Malathion lotion 0.5% (applied and washed off after 8-12 hours) or oral ivermectin (250 µg/kg, repeated after 2 weeks) may be used for alternative therapies or cases of permethrin or pyrethrin resistance.⁷ Ivermectin also is effective for involvement of eyelashes where topical insecticides should not be used.⁹ Sexual partners should be treated to prevent repeat transmission.^7,8 Bedding and clothing can be decontaminated by machine wash on hot cycle or isolating from body contact for 72 hours.⁷ Patients also should be screened for other sexually transmitted infections, including human immunodeficiency virus.⁶

The Diagnosis: Pediculosis Pubis

Dermoscopy of the pubic hair demonstrated a louse clutching multiple shafts of hairs (Figure) as well as scattered nits, confirming the presence of Phthirus pubis and diagnosis of pediculosis pubis. The key clinical diagnostic feature in this case was severe itching of the pubic area, with visible nits present on pubic hairs. Itching and infestation also can involve other hair-bearing areas such as the chest, legs, and axillae. The patient was treated with permethrin cream 5% applied to the pubic area and chest. Symptoms and infestation were resolved at 1-week follow-up.

Pediculosis pubis is an infestation of pubic hairs by the pubic (crab) louse P pubis, which feeds on host blood. Other body areas covered with dense hair also may be involved; 60% of patients are infested in at least 2 different sites.¹ Pediculosis pubis is most commonly sexually transmitted through direct contact.² Worldwide prevalence has been estimated at approximately 2% of the adult population, and a survey of 817 US college students in 2009 indicated a lifetime prevalence of 1.3%.³ The prevalence is slightly higher in men, highest in men who have sex with men, and rare in individuals with shaved pubic hair.¹ The most common symptom is pruritus of the genital area. Infested patients also may develop asymptomatic bluish gray macules (maculae ceruleae) secondary to hemosiderin deposition from louse bites.⁴

The diagnosis of pediculosis pubis is made by identification of P pubis, either by examination with the naked eye or confirmation with dermoscopy or microscopy. Although the 0.8- to 1.2-mm lice are visible to the naked eye, they can be difficult to see if not filled with blood, and nits on the hairs can be mistaken for white piedra (fungal infection of the hair shafts) or trichomycosis pubis (bacterial infection of the hair shafts).⁴ Scabies and tinea cruris do not present with attachments to the hairs. Scabies may present with papules and burrows and tinea cruris with scaly erythematous plaques. Small numbers of lice and nits may be missed by the naked eye or a traditional magnifying glass.⁵ The use of dermoscopy allows for fast and accurate identification of the characteristic lice and nits, even in these more challenging cases.⁵ Accurate diagnosis is important, as approximately 31% of infested patients have other concurrent sexually transmitted infections that warrant screening.⁶

The Centers for Disease Control and Prevention recommends first-line treatment with permethrin cream (1% or 5%) or pyrethrin with piperonyl butoxide applied to all affected areas and washed off after 10 minutes.⁷ Patients should be reevaluated after 1 week if symptoms persist and re-treated if lice are found on examination.^7,8 Malathion lotion 0.5% (applied and washed off after 8-12 hours) or oral ivermectin (250 µg/kg, repeated after 2 weeks) may be used for alternative therapies or cases of permethrin or pyrethrin resistance.⁷ Ivermectin also is effective for involvement of eyelashes where topical insecticides should not be used.⁹ Sexual partners should be treated to prevent repeat transmission.^7,8 Bedding and clothing can be decontaminated by machine wash on hot cycle or isolating from body contact for 72 hours.⁷ Patients also should be screened for other sexually transmitted infections, including human immunodeficiency virus.⁶

The Diagnosis: Trichotillomania

A scalp punch biopsy revealed pigmented hair casts, an increase in catagen and telogen follicles, and a lack of perifollicular inflammation (Figure). Based on the clinical and histopathological findings, a diagnosis of trichotillomania (TTM) was established.

Trichotillomania is a hairpulling disorder with notable dermatologic and psychiatric overlap. Although previously considered an impulse control disorder, the Diagnostic and Statistical Manual of Mental Disorders (Fifth Edition) reclassified it within obsessive-compulsive and related disorders, which also include body dysmorphic disorder and excoriation (skin-picking) disorder. Diagnostic criteria for TTM include the following: the patient must have recurrent pulling out of his/her hair resulting in hair loss despite repeated attempts to stop; underlying medical conditions and other psychiatric diagnoses must be excluded; and the patient must experience distress or impairment in social, occupational, or other areas of functioning from the hairpulling.¹ Trichotillomania mainly occurs in children and young adults, with a lifetime prevalence of approximately 1% to 2%.² The coexistence of a mood or anxiety disorder is common, as seen in our patient.

The diagnosis of TTM requires strong clinical suspicion because patients and their parents/guardians usually deny hairpulling. The main clinical differential diagnosis often is alopecia areata (AA) because both conditions can present as well-defined patches of nonscarring hair loss. Trichoscopy provides an invaluable noninvasive diagnostic tool that can be particularly useful in pediatric patients who may be reluctant to have a scalp biopsy. There are many overlapping trichoscopic findings of TTM and AA, including yellow dots, black dots, broken hairs, coiled hairs, and exclamation mark hairs.³ More specific trichoscopy findings for TTM include flame hairs (wavy proximal hair residue), V-sign (2 shafts within 1 follicle broken at the same length), and tulip hairs (dark, tulip-shaped ends of broken hairs).⁴ Hair breakage of varying lengths and trichoptilosis (split ends) can be better visualized using trichoscopy and support a diagnosis of TTM over AA.

Androgenetic alopecia (female pattern hair loss) presents with gradual thinning around the part line of the frontal and parietal scalp with trichoscopy showing miniaturization of hairs and decreased follicle density. The moth-eaten-like appearance of alopecia due to secondary syphilis may mimic alopecia areata clinically, but serologic testing can confirm the diagnosis of syphilis. Telogen effluvium does not have the trichoscopic features that are seen in TTM and is clinically distinguished by hair shedding and a positive hair pull test.

Biopsy can provide objective yet nonspecific support for the diagnosis, demonstrating trichomalacia, pigmented hair casts, empty follicles, and an increase in catagen hairs with a lack of inflammation. Normal and damaged hair follicles may be seen in close proximity, and hemorrhage may be seen secondary to trauma. Pigmented hair casts are not specific to TTM and are present in other traumatic hair disorders, such as traction alopecia; therefore, clinical correlation is essential for diagnosis.

Habit reversal training is the most effective treatment of TTM and involves 3 major components: awareness training with self-monitoring, stimulus control, and competing response procedures.⁵ Although numerous pharmacotherapies have been reported as effective treatments for TTM, a 2013 Cochrane review of 8 randomized controlled trials concluded that no medication has demonstrated reliable efficacy. Reported therapies included selective serotonin reuptake inhibitors, naltrexone, olanzapine, N-acetylcysteine, and clomipramine.⁶

The Diagnosis: Trichotillomania

A scalp punch biopsy revealed pigmented hair casts, an increase in catagen and telogen follicles, and a lack of perifollicular inflammation (Figure). Based on the clinical and histopathological findings, a diagnosis of trichotillomania (TTM) was established.

Trichotillomania is a hairpulling disorder with notable dermatologic and psychiatric overlap. Although previously considered an impulse control disorder, the Diagnostic and Statistical Manual of Mental Disorders (Fifth Edition) reclassified it within obsessive-compulsive and related disorders, which also include body dysmorphic disorder and excoriation (skin-picking) disorder. Diagnostic criteria for TTM include the following: the patient must have recurrent pulling out of his/her hair resulting in hair loss despite repeated attempts to stop; underlying medical conditions and other psychiatric diagnoses must be excluded; and the patient must experience distress or impairment in social, occupational, or other areas of functioning from the hairpulling.¹ Trichotillomania mainly occurs in children and young adults, with a lifetime prevalence of approximately 1% to 2%.² The coexistence of a mood or anxiety disorder is common, as seen in our patient.

The diagnosis of TTM requires strong clinical suspicion because patients and their parents/guardians usually deny hairpulling. The main clinical differential diagnosis often is alopecia areata (AA) because both conditions can present as well-defined patches of nonscarring hair loss. Trichoscopy provides an invaluable noninvasive diagnostic tool that can be particularly useful in pediatric patients who may be reluctant to have a scalp biopsy. There are many overlapping trichoscopic findings of TTM and AA, including yellow dots, black dots, broken hairs, coiled hairs, and exclamation mark hairs.³ More specific trichoscopy findings for TTM include flame hairs (wavy proximal hair residue), V-sign (2 shafts within 1 follicle broken at the same length), and tulip hairs (dark, tulip-shaped ends of broken hairs).⁴ Hair breakage of varying lengths and trichoptilosis (split ends) can be better visualized using trichoscopy and support a diagnosis of TTM over AA.

Androgenetic alopecia (female pattern hair loss) presents with gradual thinning around the part line of the frontal and parietal scalp with trichoscopy showing miniaturization of hairs and decreased follicle density. The moth-eaten-like appearance of alopecia due to secondary syphilis may mimic alopecia areata clinically, but serologic testing can confirm the diagnosis of syphilis. Telogen effluvium does not have the trichoscopic features that are seen in TTM and is clinically distinguished by hair shedding and a positive hair pull test.

Biopsy can provide objective yet nonspecific support for the diagnosis, demonstrating trichomalacia, pigmented hair casts, empty follicles, and an increase in catagen hairs with a lack of inflammation. Normal and damaged hair follicles may be seen in close proximity, and hemorrhage may be seen secondary to trauma. Pigmented hair casts are not specific to TTM and are present in other traumatic hair disorders, such as traction alopecia; therefore, clinical correlation is essential for diagnosis.

Habit reversal training is the most effective treatment of TTM and involves 3 major components: awareness training with self-monitoring, stimulus control, and competing response procedures.⁵ Although numerous pharmacotherapies have been reported as effective treatments for TTM, a 2013 Cochrane review of 8 randomized controlled trials concluded that no medication has demonstrated reliable efficacy. Reported therapies included selective serotonin reuptake inhibitors, naltrexone, olanzapine, N-acetylcysteine, and clomipramine.⁶

The Diagnosis: Trichotillomania

A scalp punch biopsy revealed pigmented hair casts, an increase in catagen and telogen follicles, and a lack of perifollicular inflammation (Figure). Based on the clinical and histopathological findings, a diagnosis of trichotillomania (TTM) was established.

Trichotillomania is a hairpulling disorder with notable dermatologic and psychiatric overlap. Although previously considered an impulse control disorder, the Diagnostic and Statistical Manual of Mental Disorders (Fifth Edition) reclassified it within obsessive-compulsive and related disorders, which also include body dysmorphic disorder and excoriation (skin-picking) disorder. Diagnostic criteria for TTM include the following: the patient must have recurrent pulling out of his/her hair resulting in hair loss despite repeated attempts to stop; underlying medical conditions and other psychiatric diagnoses must be excluded; and the patient must experience distress or impairment in social, occupational, or other areas of functioning from the hairpulling.¹ Trichotillomania mainly occurs in children and young adults, with a lifetime prevalence of approximately 1% to 2%.² The coexistence of a mood or anxiety disorder is common, as seen in our patient.

The diagnosis of TTM requires strong clinical suspicion because patients and their parents/guardians usually deny hairpulling. The main clinical differential diagnosis often is alopecia areata (AA) because both conditions can present as well-defined patches of nonscarring hair loss. Trichoscopy provides an invaluable noninvasive diagnostic tool that can be particularly useful in pediatric patients who may be reluctant to have a scalp biopsy. There are many overlapping trichoscopic findings of TTM and AA, including yellow dots, black dots, broken hairs, coiled hairs, and exclamation mark hairs.³ More specific trichoscopy findings for TTM include flame hairs (wavy proximal hair residue), V-sign (2 shafts within 1 follicle broken at the same length), and tulip hairs (dark, tulip-shaped ends of broken hairs).⁴ Hair breakage of varying lengths and trichoptilosis (split ends) can be better visualized using trichoscopy and support a diagnosis of TTM over AA.

Androgenetic alopecia (female pattern hair loss) presents with gradual thinning around the part line of the frontal and parietal scalp with trichoscopy showing miniaturization of hairs and decreased follicle density. The moth-eaten-like appearance of alopecia due to secondary syphilis may mimic alopecia areata clinically, but serologic testing can confirm the diagnosis of syphilis. Telogen effluvium does not have the trichoscopic features that are seen in TTM and is clinically distinguished by hair shedding and a positive hair pull test.

Biopsy can provide objective yet nonspecific support for the diagnosis, demonstrating trichomalacia, pigmented hair casts, empty follicles, and an increase in catagen hairs with a lack of inflammation. Normal and damaged hair follicles may be seen in close proximity, and hemorrhage may be seen secondary to trauma. Pigmented hair casts are not specific to TTM and are present in other traumatic hair disorders, such as traction alopecia; therefore, clinical correlation is essential for diagnosis.

Habit reversal training is the most effective treatment of TTM and involves 3 major components: awareness training with self-monitoring, stimulus control, and competing response procedures.⁵ Although numerous pharmacotherapies have been reported as effective treatments for TTM, a 2013 Cochrane review of 8 randomized controlled trials concluded that no medication has demonstrated reliable efficacy. Reported therapies included selective serotonin reuptake inhibitors, naltrexone, olanzapine, N-acetylcysteine, and clomipramine.⁶

Autologous treatment with injected platelet-rich plasma (PRP) yielded substantial improvement in hair count and shaft thickness in patients with androgenetic alopecia (AGA) after three monthly treatments, in a study that compared two treatment regimens.

PRP is gaining popularity because of its efficacy in stimulating fibroblast proliferation, triggering the production of collagen and elastin, and boosting the quantity and quality of the extracellular matrix, noted the investigators, Amelia K. Hausauer, MD, in private practice in Campbell, Calif., and Derek H. Jones, MD, in private practice in Los Angeles. Both are also with the department of dermatology at the University of California, Los Angeles.

They undertook this study to determine the optimal number and timing of treatments in patients with AGA, comparing two different injection protocols over a 6-month period. The study evaluated 40 healthy men (30) and women (10), whose mean age was 44 years, with AGA stages Norwood-Hamilton II-V (in men) and Ludwig I2-II1 (in women), recruited from a private practice in Los Angeles between November 2016 and January 2017. They were randomly assigned to one of two treatment groups: three monthly sessions followed by a fourth injection 3 months later (group 1), or two treatments, one at baseline and the second 3 months later (group 2). One of the men dropped out for reasons unrelated to the treatment.

Those with clinically stable effects of Food and Drug Administration–approved AGA treatments for 12 months were permitted to participate while continuing those treatments (topical minoxidil and/or oral finasteride), since PRP is often coadministered with other therapies. But additional products, devices, or medications used for hair regrowth were not allowed. The washout period for antiandrogen therapies was 90 days.

At 3 months, the mean increase in hair counts was significant in the first group only, but at 6 months, both groups experienced significant increases in hair count (P less than .001). However, those in the first group had superior results at 6 months, with a mean 30% increase in hair counts from baseline, compared with a 7% increase in the second group (P less than .001).

Both groups had significant increases in the mean hair shaft caliber at 3 and 6 months.

Overall, 82% of participants who completed treatment reported being satisfied or highly satisfied, and 72% expressed interest in continuing treatment after the study period; almost two-thirds considered the procedure “tolerable.”

While the authors stipulated that they did not undertake the study primarily to predict treatment response to PRP, they uncovered some significant trends that they said warranted further evaluation, including the finding that those who had experienced hair loss for less than 5-6 years were more likely to have rapid and pronounced treatment response.

Their overall findings correlated with those of previous studies supporting the increase in density of hair or hair numbers, but the existing literature draws from studies that have been open label or unblinded, which makes it difficult to evaluate them head to head. The novel, subdermal injection technique used in the study “allows for fewer, more widely spaced injection points than the traditional nappage procedure ... because PRP can diffuse further once in the deeper, subgaleal space,” they wrote. The investigators noted similar response between men and women, which is important given sparse data on the efficacy of PRP in women.

Weaknesses of the study included its small sample size and short follow-up period, the authors noted. Longer-duration studies have reported relapse between 3 and 12 months.

This study is the first of its kind to directly compare efficacy rates of two injection protocols, the authors wrote, cautioning that future studies are necessary to “fine-tune preparation methods, determine optimal maintenance schedule(s), and parse out clinical predictors of efficacy.”

Eclipse Aesthetics (the manufacturer of the PRP preparation kits) provided funding for this study, but the authors acknowledged no significant interest with commercial supporters.

SOURCE: Hausauer A et al. Dermatol Surg. 2018 Sep;44(9):1191-200.

Autologous treatment with injected platelet-rich plasma (PRP) yielded substantial improvement in hair count and shaft thickness in patients with androgenetic alopecia (AGA) after three monthly treatments, in a study that compared two treatment regimens.

PRP is gaining popularity because of its efficacy in stimulating fibroblast proliferation, triggering the production of collagen and elastin, and boosting the quantity and quality of the extracellular matrix, noted the investigators, Amelia K. Hausauer, MD, in private practice in Campbell, Calif., and Derek H. Jones, MD, in private practice in Los Angeles. Both are also with the department of dermatology at the University of California, Los Angeles.

They undertook this study to determine the optimal number and timing of treatments in patients with AGA, comparing two different injection protocols over a 6-month period. The study evaluated 40 healthy men (30) and women (10), whose mean age was 44 years, with AGA stages Norwood-Hamilton II-V (in men) and Ludwig I2-II1 (in women), recruited from a private practice in Los Angeles between November 2016 and January 2017. They were randomly assigned to one of two treatment groups: three monthly sessions followed by a fourth injection 3 months later (group 1), or two treatments, one at baseline and the second 3 months later (group 2). One of the men dropped out for reasons unrelated to the treatment.

Those with clinically stable effects of Food and Drug Administration–approved AGA treatments for 12 months were permitted to participate while continuing those treatments (topical minoxidil and/or oral finasteride), since PRP is often coadministered with other therapies. But additional products, devices, or medications used for hair regrowth were not allowed. The washout period for antiandrogen therapies was 90 days.

At 3 months, the mean increase in hair counts was significant in the first group only, but at 6 months, both groups experienced significant increases in hair count (P less than .001). However, those in the first group had superior results at 6 months, with a mean 30% increase in hair counts from baseline, compared with a 7% increase in the second group (P less than .001).

Both groups had significant increases in the mean hair shaft caliber at 3 and 6 months.

Overall, 82% of participants who completed treatment reported being satisfied or highly satisfied, and 72% expressed interest in continuing treatment after the study period; almost two-thirds considered the procedure “tolerable.”

While the authors stipulated that they did not undertake the study primarily to predict treatment response to PRP, they uncovered some significant trends that they said warranted further evaluation, including the finding that those who had experienced hair loss for less than 5-6 years were more likely to have rapid and pronounced treatment response.

Their overall findings correlated with those of previous studies supporting the increase in density of hair or hair numbers, but the existing literature draws from studies that have been open label or unblinded, which makes it difficult to evaluate them head to head. The novel, subdermal injection technique used in the study “allows for fewer, more widely spaced injection points than the traditional nappage procedure ... because PRP can diffuse further once in the deeper, subgaleal space,” they wrote. The investigators noted similar response between men and women, which is important given sparse data on the efficacy of PRP in women.

Weaknesses of the study included its small sample size and short follow-up period, the authors noted. Longer-duration studies have reported relapse between 3 and 12 months.

This study is the first of its kind to directly compare efficacy rates of two injection protocols, the authors wrote, cautioning that future studies are necessary to “fine-tune preparation methods, determine optimal maintenance schedule(s), and parse out clinical predictors of efficacy.”

Eclipse Aesthetics (the manufacturer of the PRP preparation kits) provided funding for this study, but the authors acknowledged no significant interest with commercial supporters.

SOURCE: Hausauer A et al. Dermatol Surg. 2018 Sep;44(9):1191-200.

Autologous treatment with injected platelet-rich plasma (PRP) yielded substantial improvement in hair count and shaft thickness in patients with androgenetic alopecia (AGA) after three monthly treatments, in a study that compared two treatment regimens.

PRP is gaining popularity because of its efficacy in stimulating fibroblast proliferation, triggering the production of collagen and elastin, and boosting the quantity and quality of the extracellular matrix, noted the investigators, Amelia K. Hausauer, MD, in private practice in Campbell, Calif., and Derek H. Jones, MD, in private practice in Los Angeles. Both are also with the department of dermatology at the University of California, Los Angeles.

They undertook this study to determine the optimal number and timing of treatments in patients with AGA, comparing two different injection protocols over a 6-month period. The study evaluated 40 healthy men (30) and women (10), whose mean age was 44 years, with AGA stages Norwood-Hamilton II-V (in men) and Ludwig I2-II1 (in women), recruited from a private practice in Los Angeles between November 2016 and January 2017. They were randomly assigned to one of two treatment groups: three monthly sessions followed by a fourth injection 3 months later (group 1), or two treatments, one at baseline and the second 3 months later (group 2). One of the men dropped out for reasons unrelated to the treatment.

Those with clinically stable effects of Food and Drug Administration–approved AGA treatments for 12 months were permitted to participate while continuing those treatments (topical minoxidil and/or oral finasteride), since PRP is often coadministered with other therapies. But additional products, devices, or medications used for hair regrowth were not allowed. The washout period for antiandrogen therapies was 90 days.

At 3 months, the mean increase in hair counts was significant in the first group only, but at 6 months, both groups experienced significant increases in hair count (P less than .001). However, those in the first group had superior results at 6 months, with a mean 30% increase in hair counts from baseline, compared with a 7% increase in the second group (P less than .001).

Both groups had significant increases in the mean hair shaft caliber at 3 and 6 months.

Overall, 82% of participants who completed treatment reported being satisfied or highly satisfied, and 72% expressed interest in continuing treatment after the study period; almost two-thirds considered the procedure “tolerable.”

While the authors stipulated that they did not undertake the study primarily to predict treatment response to PRP, they uncovered some significant trends that they said warranted further evaluation, including the finding that those who had experienced hair loss for less than 5-6 years were more likely to have rapid and pronounced treatment response.

Their overall findings correlated with those of previous studies supporting the increase in density of hair or hair numbers, but the existing literature draws from studies that have been open label or unblinded, which makes it difficult to evaluate them head to head. The novel, subdermal injection technique used in the study “allows for fewer, more widely spaced injection points than the traditional nappage procedure ... because PRP can diffuse further once in the deeper, subgaleal space,” they wrote. The investigators noted similar response between men and women, which is important given sparse data on the efficacy of PRP in women.

Weaknesses of the study included its small sample size and short follow-up period, the authors noted. Longer-duration studies have reported relapse between 3 and 12 months.

This study is the first of its kind to directly compare efficacy rates of two injection protocols, the authors wrote, cautioning that future studies are necessary to “fine-tune preparation methods, determine optimal maintenance schedule(s), and parse out clinical predictors of efficacy.”

Eclipse Aesthetics (the manufacturer of the PRP preparation kits) provided funding for this study, but the authors acknowledged no significant interest with commercial supporters.

SOURCE: Hausauer A et al. Dermatol Surg. 2018 Sep;44(9):1191-200.

PARIS – Each of two oral Janus kinase inhibitors individually showed impressive efficacy and acceptable safety and tolerability for treatment of chronic moderate to severe alopecia areata in a phase 2 study that promises to alter the therapeutic landscape in this challenging disease, Rodney Sinclair, MD, reported at the annual congress of the European Academy of Dermatology and Venereology.

Bruce Jancin/MDedge News

“My personal view is that these results represent a paradigm shift in the treatment of alopecia areata. Perhaps a line in the sand. On the one side, clinical observation, case reports, and small case series, but no reliably effective treatment. And on the other side of the line, investigational new drugs specifically targeting the pathogenesis, tested in prospective, multicenter, double-blind, placebo-controlled studies – and perhaps the first instance of evidence-based medicine arriving for alopecia areata. And I think that our patients with alopecia areata now have reason to be optimistic,” the dermatologist said.

“No reliably effective therapies now exist for alopecia areata, especially for patients with chronic extensive disease. There is an unmet need for a reliably effective therapy with a benefit-to-risk ratio that is appropriate for long-term use,” he added.

The phase 2 multicenter, placebo-controlled, double-blind study randomized 142 patients with chronic alopecia of at least 6 months duration and 50% or greater hair loss at baseline to an oral Janus kinase 3 (JAK3) inhibitor known for now as PF-06651600, an oral tyrosine kinase 2(TYK2)/JAK1 inhibitor known as PF-06700841, or placebo for 24 weeks. The first 4 weeks of the double-blind trial involved induction dosing of the JAK3 inhibitor at 200 mg per day and the TYK2/JAK 1 inhibitor at 60 mg per day. Thereafter, participants switched to maintenance dosing at 50 mg and 30 mg per day, respectively.

Participants had a median 2.3 years of disease duration; 62 patients had alopecia totalis, and 42 had alopecia universalis.

The primary efficacy endpoint was the mean change from baseline to week 24 in the Severity of Alopecia Tool (SALT) score, a well-validated metric widely utilized in hair loss studies. The mean baseline score was 88.1. At week 24, the score had dropped by a mean of 33.6% in the JAK 3 inhibitor group, 49.5% in the TYK2/JAK1 inhibitor group, and zero in placebo-treated controls.

A key secondary endpoint known as the SALT 30, reflecting at least 30% improvement in SALT score, was achieved in 47.9% of patients on the oral JAK 3 inhibitor, 59.6% of those on the TYK2/JAK1 inhibitor, and by no one in the control group. The utter unresponsiveness of placebo-treated controls confirms the investigators’ success in recruiting a study population with truly chronic alopecia areata, Dr. Sinclair noted.

Of note, 25% of patients on the JAK 3 inhibitor and 31.9% on the TYK2/JAK1 inhibitor achieved a SALT 90 response, and 12.5% and 12.8% reached SALT 100.

Significant improvement on the Eyelash Assessment and Eyebrow Assessment Scales occurred in 45.2% and 36.2% of the JAK 3 inhibitor group and in 60.7% and 51.7% of the TYK2/JAK 1 inhibitor group.

The study is ongoing. It’s Dr. Sinclair’s anecdotal impression that further improvement is occurring in the active treatment arms with continued therapy after 24 weeks, but that remains to be seen.

Adverse events were similar in nature and frequency in all three study arms with one notable exception: two cases of rhabdomyolysis in the TYK2/JAK 1 inhibitor group.Session chair DeDee Murrell, MD, homed in on that piece of information, pressing for further details.

“Are you concerned? Were there predictors?” asked Dr. Murrell, professor of dermatology at the University of New South Wales in Sydney.

The two affected patients had muscle pain and elevated creatinine kinase levels. “They didn’t feel particularly unwell but were withdrawn as a precaution, after which the condition quickly resolved,” Dr. Sinclair said.

The investigators were unable to identify any risk factors for rhabdomyolysis in the study population, he added.

Dr. Sinclair reported receiving research grants from and serving as a consultant to Pfizer, which sponsored the phase 2 study, as well as more than a dozen other pharmaceutical companies.

[email protected]

PARIS – Each of two oral Janus kinase inhibitors individually showed impressive efficacy and acceptable safety and tolerability for treatment of chronic moderate to severe alopecia areata in a phase 2 study that promises to alter the therapeutic landscape in this challenging disease, Rodney Sinclair, MD, reported at the annual congress of the European Academy of Dermatology and Venereology.

Bruce Jancin/MDedge News

“My personal view is that these results represent a paradigm shift in the treatment of alopecia areata. Perhaps a line in the sand. On the one side, clinical observation, case reports, and small case series, but no reliably effective treatment. And on the other side of the line, investigational new drugs specifically targeting the pathogenesis, tested in prospective, multicenter, double-blind, placebo-controlled studies – and perhaps the first instance of evidence-based medicine arriving for alopecia areata. And I think that our patients with alopecia areata now have reason to be optimistic,” the dermatologist said.

“No reliably effective therapies now exist for alopecia areata, especially for patients with chronic extensive disease. There is an unmet need for a reliably effective therapy with a benefit-to-risk ratio that is appropriate for long-term use,” he added.

The phase 2 multicenter, placebo-controlled, double-blind study randomized 142 patients with chronic alopecia of at least 6 months duration and 50% or greater hair loss at baseline to an oral Janus kinase 3 (JAK3) inhibitor known for now as PF-06651600, an oral tyrosine kinase 2(TYK2)/JAK1 inhibitor known as PF-06700841, or placebo for 24 weeks. The first 4 weeks of the double-blind trial involved induction dosing of the JAK3 inhibitor at 200 mg per day and the TYK2/JAK 1 inhibitor at 60 mg per day. Thereafter, participants switched to maintenance dosing at 50 mg and 30 mg per day, respectively.

Participants had a median 2.3 years of disease duration; 62 patients had alopecia totalis, and 42 had alopecia universalis.

The primary efficacy endpoint was the mean change from baseline to week 24 in the Severity of Alopecia Tool (SALT) score, a well-validated metric widely utilized in hair loss studies. The mean baseline score was 88.1. At week 24, the score had dropped by a mean of 33.6% in the JAK 3 inhibitor group, 49.5% in the TYK2/JAK1 inhibitor group, and zero in placebo-treated controls.

A key secondary endpoint known as the SALT 30, reflecting at least 30% improvement in SALT score, was achieved in 47.9% of patients on the oral JAK 3 inhibitor, 59.6% of those on the TYK2/JAK1 inhibitor, and by no one in the control group. The utter unresponsiveness of placebo-treated controls confirms the investigators’ success in recruiting a study population with truly chronic alopecia areata, Dr. Sinclair noted.

Of note, 25% of patients on the JAK 3 inhibitor and 31.9% on the TYK2/JAK1 inhibitor achieved a SALT 90 response, and 12.5% and 12.8% reached SALT 100.

Significant improvement on the Eyelash Assessment and Eyebrow Assessment Scales occurred in 45.2% and 36.2% of the JAK 3 inhibitor group and in 60.7% and 51.7% of the TYK2/JAK 1 inhibitor group.

The study is ongoing. It’s Dr. Sinclair’s anecdotal impression that further improvement is occurring in the active treatment arms with continued therapy after 24 weeks, but that remains to be seen.

Adverse events were similar in nature and frequency in all three study arms with one notable exception: two cases of rhabdomyolysis in the TYK2/JAK 1 inhibitor group.Session chair DeDee Murrell, MD, homed in on that piece of information, pressing for further details.

“Are you concerned? Were there predictors?” asked Dr. Murrell, professor of dermatology at the University of New South Wales in Sydney.

The two affected patients had muscle pain and elevated creatinine kinase levels. “They didn’t feel particularly unwell but were withdrawn as a precaution, after which the condition quickly resolved,” Dr. Sinclair said.

The investigators were unable to identify any risk factors for rhabdomyolysis in the study population, he added.

Dr. Sinclair reported receiving research grants from and serving as a consultant to Pfizer, which sponsored the phase 2 study, as well as more than a dozen other pharmaceutical companies.

[email protected]

PARIS – Each of two oral Janus kinase inhibitors individually showed impressive efficacy and acceptable safety and tolerability for treatment of chronic moderate to severe alopecia areata in a phase 2 study that promises to alter the therapeutic landscape in this challenging disease, Rodney Sinclair, MD, reported at the annual congress of the European Academy of Dermatology and Venereology.

Bruce Jancin/MDedge News

“My personal view is that these results represent a paradigm shift in the treatment of alopecia areata. Perhaps a line in the sand. On the one side, clinical observation, case reports, and small case series, but no reliably effective treatment. And on the other side of the line, investigational new drugs specifically targeting the pathogenesis, tested in prospective, multicenter, double-blind, placebo-controlled studies – and perhaps the first instance of evidence-based medicine arriving for alopecia areata. And I think that our patients with alopecia areata now have reason to be optimistic,” the dermatologist said.

“No reliably effective therapies now exist for alopecia areata, especially for patients with chronic extensive disease. There is an unmet need for a reliably effective therapy with a benefit-to-risk ratio that is appropriate for long-term use,” he added.

The phase 2 multicenter, placebo-controlled, double-blind study randomized 142 patients with chronic alopecia of at least 6 months duration and 50% or greater hair loss at baseline to an oral Janus kinase 3 (JAK3) inhibitor known for now as PF-06651600, an oral tyrosine kinase 2(TYK2)/JAK1 inhibitor known as PF-06700841, or placebo for 24 weeks. The first 4 weeks of the double-blind trial involved induction dosing of the JAK3 inhibitor at 200 mg per day and the TYK2/JAK 1 inhibitor at 60 mg per day. Thereafter, participants switched to maintenance dosing at 50 mg and 30 mg per day, respectively.

Participants had a median 2.3 years of disease duration; 62 patients had alopecia totalis, and 42 had alopecia universalis.

The primary efficacy endpoint was the mean change from baseline to week 24 in the Severity of Alopecia Tool (SALT) score, a well-validated metric widely utilized in hair loss studies. The mean baseline score was 88.1. At week 24, the score had dropped by a mean of 33.6% in the JAK 3 inhibitor group, 49.5% in the TYK2/JAK1 inhibitor group, and zero in placebo-treated controls.

A key secondary endpoint known as the SALT 30, reflecting at least 30% improvement in SALT score, was achieved in 47.9% of patients on the oral JAK 3 inhibitor, 59.6% of those on the TYK2/JAK1 inhibitor, and by no one in the control group. The utter unresponsiveness of placebo-treated controls confirms the investigators’ success in recruiting a study population with truly chronic alopecia areata, Dr. Sinclair noted.

Of note, 25% of patients on the JAK 3 inhibitor and 31.9% on the TYK2/JAK1 inhibitor achieved a SALT 90 response, and 12.5% and 12.8% reached SALT 100.

Significant improvement on the Eyelash Assessment and Eyebrow Assessment Scales occurred in 45.2% and 36.2% of the JAK 3 inhibitor group and in 60.7% and 51.7% of the TYK2/JAK 1 inhibitor group.

The study is ongoing. It’s Dr. Sinclair’s anecdotal impression that further improvement is occurring in the active treatment arms with continued therapy after 24 weeks, but that remains to be seen.

Adverse events were similar in nature and frequency in all three study arms with one notable exception: two cases of rhabdomyolysis in the TYK2/JAK 1 inhibitor group.Session chair DeDee Murrell, MD, homed in on that piece of information, pressing for further details.

“Are you concerned? Were there predictors?” asked Dr. Murrell, professor of dermatology at the University of New South Wales in Sydney.

The two affected patients had muscle pain and elevated creatinine kinase levels. “They didn’t feel particularly unwell but were withdrawn as a precaution, after which the condition quickly resolved,” Dr. Sinclair said.

The investigators were unable to identify any risk factors for rhabdomyolysis in the study population, he added.

Dr. Sinclair reported receiving research grants from and serving as a consultant to Pfizer, which sponsored the phase 2 study, as well as more than a dozen other pharmaceutical companies.

[email protected]