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Novel oral antifungal headed to phase III for onychomycosis

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– A once-weekly oral antifungal drug known as VT-1161 will move into phase III clinical testing in 2017 based on its impressive performance in an interim analysis of a phase IIb study, Amir Tavakkol, PhD, reported at the annual congress of the European Academy of Dermatology and Venereology.

“We saw robust evidence of clinical and mycologic efficacy in patients with moderate to severe onychomycosis. This was true even in patients with nails considered very difficult to treat because of dermatophytomas and spikes, which are usually poor prognostic elements,” said Dr. Tavakkol, chief development officer at Viamet Pharmaceuticals in Durham, N.C., which is developing VT-1161.

Bruce Jancin/Frontline Medical News
Dr. Amir Tavakkol
“We believe its high potency, broad therapeutic index, and favorable oral pharmacokinetics that allow a very rapid increase in exposure, followed by maintenance exposure with once-weekly dosing, highlight VT-1161’s potential for onychomycosis,” he said.

He reported on 107 patients with toenail onychomycosis who enrolled in the phase IIb, double-blind, placebo-controlled RENOVATE (Restoring Nail: An Oral VT-1161 Tablet Evaluation) study. At enrollment they averaged 47% disease involvement of the big toenail, the target nail for the trial. Participants in the five study arms had an average of 4.2-5.0 affected toenails. Both the percentage of nail involvement and the number of diseased toenails were roughly twice as great as is typical in studies of topical antifungals, underscoring that participants in the VT-1161 trial had fairly severe onychomycosis.

Patients were assigned to one of five study arms: 300 mg of VT-1161 once weekly for 12 weeks, then 12 weeks of placebo; 600 mg of VT-1161 once weekly for 12 weeks, followed by 12 weeks of placebo; either 300 or 600 mg of the antifungal agent once weekly for the full 24 weeks; or 24 weeks of once-per-week placebo. Immediately prior to the formal start of the study, however, everyone received 14 days of a once-daily loading dose of VT-1161 or placebo at the dose they would subsequently take once weekly during the trial.

The primary outcome in the ongoing study is the percentage of patients with a complete cure, both mycologic and clinical, at 48 weeks. Those data aren’t in yet, but Dr. Tavakkol presented the results of the prespecified interim analysis at 24 weeks. He drew attention to the substantial percentage of patients with no more than 10% nail involvement after either 12 or 24 weeks of once-weekly VT-1161.

“Please keep in mind that this is only at most 24 weeks of therapy. Given the rate of nail growth at 1 mm per month when it is healthy, these are remarkable data. Ten percent or less nail involvement is basically 1-2 mm at the distal end. I believe a substantial percentage of these patients will reach clinical cure by 48 weeks,” he said.

VT-1161, a molecule with high selectivity for fungal CYP51, blocks the production of ergosterol, a key component of the fungal cell membrane, according to the company. It has no known drug interactions. At all doses studied in the trial, it was safe and well tolerated, with no abnormal liver function tests, no effect on bilirubin, and no change in QTc interval. Only 8 of the 107 patients reported adverse events deemed possibly related to the study drug by blinded investigators. No one dropped out of the study.

“VT-1161 is also being developed for recurrent vulvovaginal candidiasis. The results there are outstanding, too,” Dr. Tavakkol said.

The trial was funded by Viamet, where he is employed.
 
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– A once-weekly oral antifungal drug known as VT-1161 will move into phase III clinical testing in 2017 based on its impressive performance in an interim analysis of a phase IIb study, Amir Tavakkol, PhD, reported at the annual congress of the European Academy of Dermatology and Venereology.

“We saw robust evidence of clinical and mycologic efficacy in patients with moderate to severe onychomycosis. This was true even in patients with nails considered very difficult to treat because of dermatophytomas and spikes, which are usually poor prognostic elements,” said Dr. Tavakkol, chief development officer at Viamet Pharmaceuticals in Durham, N.C., which is developing VT-1161.

Bruce Jancin/Frontline Medical News
Dr. Amir Tavakkol
“We believe its high potency, broad therapeutic index, and favorable oral pharmacokinetics that allow a very rapid increase in exposure, followed by maintenance exposure with once-weekly dosing, highlight VT-1161’s potential for onychomycosis,” he said.

He reported on 107 patients with toenail onychomycosis who enrolled in the phase IIb, double-blind, placebo-controlled RENOVATE (Restoring Nail: An Oral VT-1161 Tablet Evaluation) study. At enrollment they averaged 47% disease involvement of the big toenail, the target nail for the trial. Participants in the five study arms had an average of 4.2-5.0 affected toenails. Both the percentage of nail involvement and the number of diseased toenails were roughly twice as great as is typical in studies of topical antifungals, underscoring that participants in the VT-1161 trial had fairly severe onychomycosis.

Patients were assigned to one of five study arms: 300 mg of VT-1161 once weekly for 12 weeks, then 12 weeks of placebo; 600 mg of VT-1161 once weekly for 12 weeks, followed by 12 weeks of placebo; either 300 or 600 mg of the antifungal agent once weekly for the full 24 weeks; or 24 weeks of once-per-week placebo. Immediately prior to the formal start of the study, however, everyone received 14 days of a once-daily loading dose of VT-1161 or placebo at the dose they would subsequently take once weekly during the trial.

The primary outcome in the ongoing study is the percentage of patients with a complete cure, both mycologic and clinical, at 48 weeks. Those data aren’t in yet, but Dr. Tavakkol presented the results of the prespecified interim analysis at 24 weeks. He drew attention to the substantial percentage of patients with no more than 10% nail involvement after either 12 or 24 weeks of once-weekly VT-1161.

“Please keep in mind that this is only at most 24 weeks of therapy. Given the rate of nail growth at 1 mm per month when it is healthy, these are remarkable data. Ten percent or less nail involvement is basically 1-2 mm at the distal end. I believe a substantial percentage of these patients will reach clinical cure by 48 weeks,” he said.

VT-1161, a molecule with high selectivity for fungal CYP51, blocks the production of ergosterol, a key component of the fungal cell membrane, according to the company. It has no known drug interactions. At all doses studied in the trial, it was safe and well tolerated, with no abnormal liver function tests, no effect on bilirubin, and no change in QTc interval. Only 8 of the 107 patients reported adverse events deemed possibly related to the study drug by blinded investigators. No one dropped out of the study.

“VT-1161 is also being developed for recurrent vulvovaginal candidiasis. The results there are outstanding, too,” Dr. Tavakkol said.

The trial was funded by Viamet, where he is employed.
 

 

– A once-weekly oral antifungal drug known as VT-1161 will move into phase III clinical testing in 2017 based on its impressive performance in an interim analysis of a phase IIb study, Amir Tavakkol, PhD, reported at the annual congress of the European Academy of Dermatology and Venereology.

“We saw robust evidence of clinical and mycologic efficacy in patients with moderate to severe onychomycosis. This was true even in patients with nails considered very difficult to treat because of dermatophytomas and spikes, which are usually poor prognostic elements,” said Dr. Tavakkol, chief development officer at Viamet Pharmaceuticals in Durham, N.C., which is developing VT-1161.

Bruce Jancin/Frontline Medical News
Dr. Amir Tavakkol
“We believe its high potency, broad therapeutic index, and favorable oral pharmacokinetics that allow a very rapid increase in exposure, followed by maintenance exposure with once-weekly dosing, highlight VT-1161’s potential for onychomycosis,” he said.

He reported on 107 patients with toenail onychomycosis who enrolled in the phase IIb, double-blind, placebo-controlled RENOVATE (Restoring Nail: An Oral VT-1161 Tablet Evaluation) study. At enrollment they averaged 47% disease involvement of the big toenail, the target nail for the trial. Participants in the five study arms had an average of 4.2-5.0 affected toenails. Both the percentage of nail involvement and the number of diseased toenails were roughly twice as great as is typical in studies of topical antifungals, underscoring that participants in the VT-1161 trial had fairly severe onychomycosis.

Patients were assigned to one of five study arms: 300 mg of VT-1161 once weekly for 12 weeks, then 12 weeks of placebo; 600 mg of VT-1161 once weekly for 12 weeks, followed by 12 weeks of placebo; either 300 or 600 mg of the antifungal agent once weekly for the full 24 weeks; or 24 weeks of once-per-week placebo. Immediately prior to the formal start of the study, however, everyone received 14 days of a once-daily loading dose of VT-1161 or placebo at the dose they would subsequently take once weekly during the trial.

The primary outcome in the ongoing study is the percentage of patients with a complete cure, both mycologic and clinical, at 48 weeks. Those data aren’t in yet, but Dr. Tavakkol presented the results of the prespecified interim analysis at 24 weeks. He drew attention to the substantial percentage of patients with no more than 10% nail involvement after either 12 or 24 weeks of once-weekly VT-1161.

“Please keep in mind that this is only at most 24 weeks of therapy. Given the rate of nail growth at 1 mm per month when it is healthy, these are remarkable data. Ten percent or less nail involvement is basically 1-2 mm at the distal end. I believe a substantial percentage of these patients will reach clinical cure by 48 weeks,” he said.

VT-1161, a molecule with high selectivity for fungal CYP51, blocks the production of ergosterol, a key component of the fungal cell membrane, according to the company. It has no known drug interactions. At all doses studied in the trial, it was safe and well tolerated, with no abnormal liver function tests, no effect on bilirubin, and no change in QTc interval. Only 8 of the 107 patients reported adverse events deemed possibly related to the study drug by blinded investigators. No one dropped out of the study.

“VT-1161 is also being developed for recurrent vulvovaginal candidiasis. The results there are outstanding, too,” Dr. Tavakkol said.

The trial was funded by Viamet, where he is employed.
 
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Key clinical point: A novel once-weekly oral antifungal drug known as VT-1161 is headed for phase III studies after an impressive performance in a phase IIb trial.

Major finding: Among onychomycosis patients with an average 47% target toenail involvement at baseline, 35% had improved to no more than 10% nail involvement at 24 weeks after 12 weeks of once-weekly VT-1161 followed by 12 weeks of placebo.

Data source: A double-blind, randomized, phase IIb clinical trial involving 107 patients with toenail onychomycosis.

Disclosures: The trial was funded by Viamet Pharmaceuticals, where the study presenter is employed.

Survey finds high rate of misdiagnosed fungal infections

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Fungal skin infections may be missed or misdiagnosed by many dermatologists, according to the results of a survey published online in the Journal of the American Academy of Dermatology.

Dr. Adam Friedman
One of the authors, Adam Friedman, MD, was not surprised by the results.

“Dermatology is full of doppelgangers,” Dr. Friedman, director of the residency program and of translational research in the department of dermatology at George Washington University, said in an interview. 

“While we [dermatologists] pride ourselves on our visual prowess, there are many skin diseases which do not follow the textbook and can be quite protean in their presentations,” he said.

The variability in presentation makes diagnosing fungal infections especially challenging, he noted. “Fungal infections of the skin can have many clinical flavors and can infect skin, hair and nails. Also, inappropriate treatment can obscure the appearance of the infection, and the fact that there are multiple other conditions that can look like these [fungal] infections makes proper identification difficult.”

Although the results were limited by several factors including possible selection bias, lack of measurable response rate, and small sample size, the findings highlight how easy it can be to miss a diagnosis of fungal infection, “which can result in inappropriate therapy, worsening of symptoms, and even additional skin and soft-tissue infections,” the researchers wrote.

“Keep an open mind and cast a wider differential,” to help catch fungal infections, and use all the dermatologic tools, including slide preps, cultures, and biopsies, Dr. Friedman said. Better diagnostic tools and improved training for clinicians outside of dermatology also could reduce the misdiagnosis of fungal infections, he added. “Many of these patients are misdiagnosed in the emergency department, urgent care, or primary care settings,” and delayed treatment increases associated morbidity, he said.

Mr. Yadgar, Dr. Friedman, and another coauthor, Neal Bhatia, MD, of Therapeutics Clinical Research, San Diego, Calif., had no financial conflicts to disclose. There was no funding source.
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Fungal skin infections may be missed or misdiagnosed by many dermatologists, according to the results of a survey published online in the Journal of the American Academy of Dermatology.

Dr. Adam Friedman
One of the authors, Adam Friedman, MD, was not surprised by the results.

“Dermatology is full of doppelgangers,” Dr. Friedman, director of the residency program and of translational research in the department of dermatology at George Washington University, said in an interview. 

“While we [dermatologists] pride ourselves on our visual prowess, there are many skin diseases which do not follow the textbook and can be quite protean in their presentations,” he said.

The variability in presentation makes diagnosing fungal infections especially challenging, he noted. “Fungal infections of the skin can have many clinical flavors and can infect skin, hair and nails. Also, inappropriate treatment can obscure the appearance of the infection, and the fact that there are multiple other conditions that can look like these [fungal] infections makes proper identification difficult.”

Although the results were limited by several factors including possible selection bias, lack of measurable response rate, and small sample size, the findings highlight how easy it can be to miss a diagnosis of fungal infection, “which can result in inappropriate therapy, worsening of symptoms, and even additional skin and soft-tissue infections,” the researchers wrote.

“Keep an open mind and cast a wider differential,” to help catch fungal infections, and use all the dermatologic tools, including slide preps, cultures, and biopsies, Dr. Friedman said. Better diagnostic tools and improved training for clinicians outside of dermatology also could reduce the misdiagnosis of fungal infections, he added. “Many of these patients are misdiagnosed in the emergency department, urgent care, or primary care settings,” and delayed treatment increases associated morbidity, he said.

Mr. Yadgar, Dr. Friedman, and another coauthor, Neal Bhatia, MD, of Therapeutics Clinical Research, San Diego, Calif., had no financial conflicts to disclose. There was no funding source.

 

Fungal skin infections may be missed or misdiagnosed by many dermatologists, according to the results of a survey published online in the Journal of the American Academy of Dermatology.

Dr. Adam Friedman
One of the authors, Adam Friedman, MD, was not surprised by the results.

“Dermatology is full of doppelgangers,” Dr. Friedman, director of the residency program and of translational research in the department of dermatology at George Washington University, said in an interview. 

“While we [dermatologists] pride ourselves on our visual prowess, there are many skin diseases which do not follow the textbook and can be quite protean in their presentations,” he said.

The variability in presentation makes diagnosing fungal infections especially challenging, he noted. “Fungal infections of the skin can have many clinical flavors and can infect skin, hair and nails. Also, inappropriate treatment can obscure the appearance of the infection, and the fact that there are multiple other conditions that can look like these [fungal] infections makes proper identification difficult.”

Although the results were limited by several factors including possible selection bias, lack of measurable response rate, and small sample size, the findings highlight how easy it can be to miss a diagnosis of fungal infection, “which can result in inappropriate therapy, worsening of symptoms, and even additional skin and soft-tissue infections,” the researchers wrote.

“Keep an open mind and cast a wider differential,” to help catch fungal infections, and use all the dermatologic tools, including slide preps, cultures, and biopsies, Dr. Friedman said. Better diagnostic tools and improved training for clinicians outside of dermatology also could reduce the misdiagnosis of fungal infections, he added. “Many of these patients are misdiagnosed in the emergency department, urgent care, or primary care settings,” and delayed treatment increases associated morbidity, he said.

Mr. Yadgar, Dr. Friedman, and another coauthor, Neal Bhatia, MD, of Therapeutics Clinical Research, San Diego, Calif., had no financial conflicts to disclose. There was no funding source.
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Key clinical point: Fungal infections may often be missed or misdiagnosed by dermatologists.

Major finding: In 1 of 13 cases did 90% of an audience of dermatologists correctly categorize the condition.

Data source: A survey of board-certified dermatologists, asked whether or not 13 clinical images were a fungal infection or not, during a session on fungal infections at a dermatology meeting.

Disclosures: The research team had no relevant financial conflicts to disclose.

VIDEO: Topical antifungals win with patients

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New topical treatment options for onychomycosis represent significant improvements over older agents, and may approach the success seen with oral drugs, according to Dr. Theodore Rosen, professor of dermatology, Baylor College of Medicine, Houston.

Efinaconazole and tavaborole both permeate the nail and allow for spreading to the lateral nail folds and hyponychium, Dr. Rosen said in a video interview at the Skin Disease Education Foundation’s annual Las Vegas Dermatology Seminar. Moreover, the topical treatments are popular with patients. Even if patients are not 100% clear, they are usually happy if their condition improves enough to wear sandals with confidence, Dr. Rosen added in the interview.

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

Dr. Rosen disclosed being a paid participant on the scientific advisory boards for Anacor and Valeant.

The video associated with this article is no longer available on this site. Please view all of our videos on the MDedge YouTube channel
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New topical treatment options for onychomycosis represent significant improvements over older agents, and may approach the success seen with oral drugs, according to Dr. Theodore Rosen, professor of dermatology, Baylor College of Medicine, Houston.

Efinaconazole and tavaborole both permeate the nail and allow for spreading to the lateral nail folds and hyponychium, Dr. Rosen said in a video interview at the Skin Disease Education Foundation’s annual Las Vegas Dermatology Seminar. Moreover, the topical treatments are popular with patients. Even if patients are not 100% clear, they are usually happy if their condition improves enough to wear sandals with confidence, Dr. Rosen added in the interview.

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

Dr. Rosen disclosed being a paid participant on the scientific advisory boards for Anacor and Valeant.

The video associated with this article is no longer available on this site. Please view all of our videos on the MDedge YouTube channel

New topical treatment options for onychomycosis represent significant improvements over older agents, and may approach the success seen with oral drugs, according to Dr. Theodore Rosen, professor of dermatology, Baylor College of Medicine, Houston.

Efinaconazole and tavaborole both permeate the nail and allow for spreading to the lateral nail folds and hyponychium, Dr. Rosen said in a video interview at the Skin Disease Education Foundation’s annual Las Vegas Dermatology Seminar. Moreover, the topical treatments are popular with patients. Even if patients are not 100% clear, they are usually happy if their condition improves enough to wear sandals with confidence, Dr. Rosen added in the interview.

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

Dr. Rosen disclosed being a paid participant on the scientific advisory boards for Anacor and Valeant.

The video associated with this article is no longer available on this site. Please view all of our videos on the MDedge YouTube channel
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Accuracy and Sources of Images From Direct Google Image Searches for Common Dermatology Terms

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Accuracy and Sources of Images From Direct Google Image Searches for Common Dermatology Terms

To the Editor:

Prior studies have assessed the quality of text-based dermatology information on the Internet using traditional search engine queries.1 However, little is understood about the sources, accuracy, and quality of online dermatology images derived from direct image searches. Previous work has shown that direct search engine image queries were largely accurate for 3 pediatric dermatology diagnosis searches: atopic dermatitis, lichen striatus, and subcutaneous fat necrosis.2 We assessed images obtained for common dermatologic conditions from a Google image search (GIS) compared to a traditional text-based Google web search (GWS).

Image results for 32 unique dermatologic search terms were analyzed (Table 1). These search terms were selected using the results of a prior study that identified the most common dermatologic diagnoses that led users to the 2 most popular dermatology-specific websites worldwide: the American Academy of Dermatology (www.aad.org) and DermNet New Zealand (www.dermnetnz.org).3 The Alexa directory (www.alexa.com), a large publicly available Internet analytics resource, was used to determine the most common dermatology search terms that led a user to either www.dermnetnz.org or www.aad.org. In addition, searches for the 3 most common types of skin cancer—melanoma, squamous cell carcinoma, and basal cell carcinoma—were included. Each term was entered into a GIS and a GWS. The first 10 results, which represent 92% of the websites ultimately visited by users,4 were analyzed. The source, diagnostic accuracy, and Fitzpatrick skin type of the images was determined. Website sources were organized into 11 categories. All data collection occurred within a 1-week period in August 2015.

A total of 320 images were analyzed. In the GIS, private websites (36%), dermatology association websites (28%), and general health information websites (10%) were the 3 most common sources. In the GWS, health information websites (35%), private websites (21%), and dermatology association websites (20%) accounted for the most common sources (Table 2). The majority of images were of Fitzpatrick skin types I and II (89%) and nearly all images were diagnostically accurate (98%). There was no statistically significant difference in accuracy of diagnosis between physician-associated websites (100% accuracy) versus nonphysician-associated sites (98% accuracy, P=.25).

Our results showed high diagnostic accuracy among the top GIS results for common dermatology search terms. Diagnostic accuracy did not vary between websites that were physician associated versus those that were not. Our results are comparable to the reported accuracy of online dermatologic health information.1 In GIS results, the majority of images were provided by private websites, whereas the top websites in GWS results were health information websites.

Only 1% of images were of Fitzpatrick skin types VI and VII. Presentation of skin diseases is remarkably different based on the patient’s skin type.5 The shortage of readily accessible images of skin of color is in line with the lack of familiarity physicians and trainees have with dermatologic conditions in ethnic skin.6

Based on the results from this analysis, providers and patients searching for dermatologic conditions via a direct GIS should be cognizant of several considerations. Although our results showed that GIS was accurate, the searcher should note that image-based searches are not accompanied by relevant text that can help confirm relevancy and accuracy. Image searches depend on textual tags added by the source website. Websites that represent dermatological associations and academic centers can add an additional layer of confidence for users. Patients and clinicians also should be aware that the consideration of a patient’s Fitzpatrick skin type is critical when assessing the relevancy of a GIS result. In conclusion, search results via GIS queries are accurate for the dermatological diagnoses tested but may be lacking in skin of color variations, suggesting a potential unmet need based on our growing ethnic skin population.

References
  1. Jensen JD, Dunnick CA, Arbuckle HA, et al. Dermatology information on the Internet: an appraisal by dermatologists and dermatology residents. J Am Acad Dermatol. 2010;63:1101-1103.
  2. Cutrone M, Grimalt R. Dermatological image search engines on the Internet: do they work? J Eur Acad Dermatol Venereol. 2007;21:175-177.
  3. Xu S, Nault A, Bhatia A. Search and engagement analysis of association websites representing dermatologists—implications and opportunities for web visibility and patient education: website rankings of dermatology associations. Pract Dermatol. In press.
  4. comScore releases July 2015 U.S. desktop search engine rankings [press release]. Reston, VA: comScore, Inc; August 14, 2015. http://www.comscore.com/Insights/Market-Rankings/comScore-Releases-July-2015-U.S.-Desktop-Search-Engine-Rankings. Accessed October 18, 2016.
  5. Kundu RV, Patterson S. Dermatologic conditions in skin of color: part I. special considerations for common skin disorders. Am Fam Physician. 2013;87:850-856.
  6. Nijhawan RI, Jacob SE, Woolery-Lloyd H. Skin of color education in dermatology residency programs: does residency training reflect the changing demographics of the United States? J Am Acad Dermatol. 2008;59:615-618.
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Author and Disclosure Information

Dr. Nault is from University of Wisconsin School of Medicine and Public Health, Madison. Drs. Bhatia and Xu are from the Department of Dermatology, Northwestern University, Feinberg School of Medicine, Chicago, Illinois. Dr. Bhatia also is from Dupage Medical Group, Naperville, Illinois.

The authors report no conflict of interest.

Correspondence: Shuai Xu, MD, MSc, 676 N St Clair St, Ste 1600, Chicago, IL 60611 ([email protected]).

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Dr. Nault is from University of Wisconsin School of Medicine and Public Health, Madison. Drs. Bhatia and Xu are from the Department of Dermatology, Northwestern University, Feinberg School of Medicine, Chicago, Illinois. Dr. Bhatia also is from Dupage Medical Group, Naperville, Illinois.

The authors report no conflict of interest.

Correspondence: Shuai Xu, MD, MSc, 676 N St Clair St, Ste 1600, Chicago, IL 60611 ([email protected]).

Author and Disclosure Information

Dr. Nault is from University of Wisconsin School of Medicine and Public Health, Madison. Drs. Bhatia and Xu are from the Department of Dermatology, Northwestern University, Feinberg School of Medicine, Chicago, Illinois. Dr. Bhatia also is from Dupage Medical Group, Naperville, Illinois.

The authors report no conflict of interest.

Correspondence: Shuai Xu, MD, MSc, 676 N St Clair St, Ste 1600, Chicago, IL 60611 ([email protected]).

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To the Editor:

Prior studies have assessed the quality of text-based dermatology information on the Internet using traditional search engine queries.1 However, little is understood about the sources, accuracy, and quality of online dermatology images derived from direct image searches. Previous work has shown that direct search engine image queries were largely accurate for 3 pediatric dermatology diagnosis searches: atopic dermatitis, lichen striatus, and subcutaneous fat necrosis.2 We assessed images obtained for common dermatologic conditions from a Google image search (GIS) compared to a traditional text-based Google web search (GWS).

Image results for 32 unique dermatologic search terms were analyzed (Table 1). These search terms were selected using the results of a prior study that identified the most common dermatologic diagnoses that led users to the 2 most popular dermatology-specific websites worldwide: the American Academy of Dermatology (www.aad.org) and DermNet New Zealand (www.dermnetnz.org).3 The Alexa directory (www.alexa.com), a large publicly available Internet analytics resource, was used to determine the most common dermatology search terms that led a user to either www.dermnetnz.org or www.aad.org. In addition, searches for the 3 most common types of skin cancer—melanoma, squamous cell carcinoma, and basal cell carcinoma—were included. Each term was entered into a GIS and a GWS. The first 10 results, which represent 92% of the websites ultimately visited by users,4 were analyzed. The source, diagnostic accuracy, and Fitzpatrick skin type of the images was determined. Website sources were organized into 11 categories. All data collection occurred within a 1-week period in August 2015.

A total of 320 images were analyzed. In the GIS, private websites (36%), dermatology association websites (28%), and general health information websites (10%) were the 3 most common sources. In the GWS, health information websites (35%), private websites (21%), and dermatology association websites (20%) accounted for the most common sources (Table 2). The majority of images were of Fitzpatrick skin types I and II (89%) and nearly all images were diagnostically accurate (98%). There was no statistically significant difference in accuracy of diagnosis between physician-associated websites (100% accuracy) versus nonphysician-associated sites (98% accuracy, P=.25).

Our results showed high diagnostic accuracy among the top GIS results for common dermatology search terms. Diagnostic accuracy did not vary between websites that were physician associated versus those that were not. Our results are comparable to the reported accuracy of online dermatologic health information.1 In GIS results, the majority of images were provided by private websites, whereas the top websites in GWS results were health information websites.

Only 1% of images were of Fitzpatrick skin types VI and VII. Presentation of skin diseases is remarkably different based on the patient’s skin type.5 The shortage of readily accessible images of skin of color is in line with the lack of familiarity physicians and trainees have with dermatologic conditions in ethnic skin.6

Based on the results from this analysis, providers and patients searching for dermatologic conditions via a direct GIS should be cognizant of several considerations. Although our results showed that GIS was accurate, the searcher should note that image-based searches are not accompanied by relevant text that can help confirm relevancy and accuracy. Image searches depend on textual tags added by the source website. Websites that represent dermatological associations and academic centers can add an additional layer of confidence for users. Patients and clinicians also should be aware that the consideration of a patient’s Fitzpatrick skin type is critical when assessing the relevancy of a GIS result. In conclusion, search results via GIS queries are accurate for the dermatological diagnoses tested but may be lacking in skin of color variations, suggesting a potential unmet need based on our growing ethnic skin population.

To the Editor:

Prior studies have assessed the quality of text-based dermatology information on the Internet using traditional search engine queries.1 However, little is understood about the sources, accuracy, and quality of online dermatology images derived from direct image searches. Previous work has shown that direct search engine image queries were largely accurate for 3 pediatric dermatology diagnosis searches: atopic dermatitis, lichen striatus, and subcutaneous fat necrosis.2 We assessed images obtained for common dermatologic conditions from a Google image search (GIS) compared to a traditional text-based Google web search (GWS).

Image results for 32 unique dermatologic search terms were analyzed (Table 1). These search terms were selected using the results of a prior study that identified the most common dermatologic diagnoses that led users to the 2 most popular dermatology-specific websites worldwide: the American Academy of Dermatology (www.aad.org) and DermNet New Zealand (www.dermnetnz.org).3 The Alexa directory (www.alexa.com), a large publicly available Internet analytics resource, was used to determine the most common dermatology search terms that led a user to either www.dermnetnz.org or www.aad.org. In addition, searches for the 3 most common types of skin cancer—melanoma, squamous cell carcinoma, and basal cell carcinoma—were included. Each term was entered into a GIS and a GWS. The first 10 results, which represent 92% of the websites ultimately visited by users,4 were analyzed. The source, diagnostic accuracy, and Fitzpatrick skin type of the images was determined. Website sources were organized into 11 categories. All data collection occurred within a 1-week period in August 2015.

A total of 320 images were analyzed. In the GIS, private websites (36%), dermatology association websites (28%), and general health information websites (10%) were the 3 most common sources. In the GWS, health information websites (35%), private websites (21%), and dermatology association websites (20%) accounted for the most common sources (Table 2). The majority of images were of Fitzpatrick skin types I and II (89%) and nearly all images were diagnostically accurate (98%). There was no statistically significant difference in accuracy of diagnosis between physician-associated websites (100% accuracy) versus nonphysician-associated sites (98% accuracy, P=.25).

Our results showed high diagnostic accuracy among the top GIS results for common dermatology search terms. Diagnostic accuracy did not vary between websites that were physician associated versus those that were not. Our results are comparable to the reported accuracy of online dermatologic health information.1 In GIS results, the majority of images were provided by private websites, whereas the top websites in GWS results were health information websites.

Only 1% of images were of Fitzpatrick skin types VI and VII. Presentation of skin diseases is remarkably different based on the patient’s skin type.5 The shortage of readily accessible images of skin of color is in line with the lack of familiarity physicians and trainees have with dermatologic conditions in ethnic skin.6

Based on the results from this analysis, providers and patients searching for dermatologic conditions via a direct GIS should be cognizant of several considerations. Although our results showed that GIS was accurate, the searcher should note that image-based searches are not accompanied by relevant text that can help confirm relevancy and accuracy. Image searches depend on textual tags added by the source website. Websites that represent dermatological associations and academic centers can add an additional layer of confidence for users. Patients and clinicians also should be aware that the consideration of a patient’s Fitzpatrick skin type is critical when assessing the relevancy of a GIS result. In conclusion, search results via GIS queries are accurate for the dermatological diagnoses tested but may be lacking in skin of color variations, suggesting a potential unmet need based on our growing ethnic skin population.

References
  1. Jensen JD, Dunnick CA, Arbuckle HA, et al. Dermatology information on the Internet: an appraisal by dermatologists and dermatology residents. J Am Acad Dermatol. 2010;63:1101-1103.
  2. Cutrone M, Grimalt R. Dermatological image search engines on the Internet: do they work? J Eur Acad Dermatol Venereol. 2007;21:175-177.
  3. Xu S, Nault A, Bhatia A. Search and engagement analysis of association websites representing dermatologists—implications and opportunities for web visibility and patient education: website rankings of dermatology associations. Pract Dermatol. In press.
  4. comScore releases July 2015 U.S. desktop search engine rankings [press release]. Reston, VA: comScore, Inc; August 14, 2015. http://www.comscore.com/Insights/Market-Rankings/comScore-Releases-July-2015-U.S.-Desktop-Search-Engine-Rankings. Accessed October 18, 2016.
  5. Kundu RV, Patterson S. Dermatologic conditions in skin of color: part I. special considerations for common skin disorders. Am Fam Physician. 2013;87:850-856.
  6. Nijhawan RI, Jacob SE, Woolery-Lloyd H. Skin of color education in dermatology residency programs: does residency training reflect the changing demographics of the United States? J Am Acad Dermatol. 2008;59:615-618.
References
  1. Jensen JD, Dunnick CA, Arbuckle HA, et al. Dermatology information on the Internet: an appraisal by dermatologists and dermatology residents. J Am Acad Dermatol. 2010;63:1101-1103.
  2. Cutrone M, Grimalt R. Dermatological image search engines on the Internet: do they work? J Eur Acad Dermatol Venereol. 2007;21:175-177.
  3. Xu S, Nault A, Bhatia A. Search and engagement analysis of association websites representing dermatologists—implications and opportunities for web visibility and patient education: website rankings of dermatology associations. Pract Dermatol. In press.
  4. comScore releases July 2015 U.S. desktop search engine rankings [press release]. Reston, VA: comScore, Inc; August 14, 2015. http://www.comscore.com/Insights/Market-Rankings/comScore-Releases-July-2015-U.S.-Desktop-Search-Engine-Rankings. Accessed October 18, 2016.
  5. Kundu RV, Patterson S. Dermatologic conditions in skin of color: part I. special considerations for common skin disorders. Am Fam Physician. 2013;87:850-856.
  6. Nijhawan RI, Jacob SE, Woolery-Lloyd H. Skin of color education in dermatology residency programs: does residency training reflect the changing demographics of the United States? J Am Acad Dermatol. 2008;59:615-618.
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  • Direct Google image searches largely deliver accurate results for common dermatological diagnoses.
  • Greater effort should be made to include more publicly available images for dermatological diseases in darker skin types.
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Scalp Psoriasis: Weighing Treatment Options

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Scalp psoriasis often is the initial presentation of psoriasis, and it can be one of the most challenging aspects of the disease. It can be difficult to treat for several reasons. First, hair can interfere with topical therapy reaching its site of action on the scalp. Second, facial skin also can be exposed to these treatments with the associated risk for adverse events. Finally, compliance often is difficult.

An evidence-based review published online on September 21 in the American Journal of Clinical Dermatology examined treatments for scalp psoriasis, including newer systemic therapies. Of 475 studies initially identified from PubMed and 845 from Embase (up to May 2016), the review included 27 clinical trials, 4 papers reporting pooled analyses of other clinical trials, 10 open-label trials, 1 case series, and 2 case reports after excluding non-English literature.

Wang and Tsai noted that few randomized controlled trials have been performed specifically in scalp psoriasis. The authors found that topical corticosteroids provide good effects and are usually recommended as first-line treatment. Calcipotriol–betamethasone dipropionate is more highly effective than either of its individual components.

The analysis also suggested that localized phototherapy is better than generalized phototherapy on hair-bearing areas. Methotrexate, cyclosporine, fumaric acid esters, and acitretin are well-recognized agents in the treatment of psoriasis, but they located no published randomized controlled trials specifically evaluating these agents in scalp psoriasis. Wang and Tsai also commented that biologics and new small-molecule agents show excellent effects on scalp psoriasis, but the high cost of these treatments mean they may be limited to use in extensive scalp psoriasis. They suggested that more controlled studies are needed for an evidence-based approach to scalp psoriasis.

What’s the issue?

Scalp psoriasis can be an isolated condition or may occur in association with more extensive disease. There has been increased attention to its treatment over the last several years, with several new options. What is your preferred approach to scalp psoriasis?

We want to know your views! Tell us what you think.

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Dr. Weinberg is from the Icahn School of Medicine at Mount Sinai, New York, New York.

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Scalp psoriasis often is the initial presentation of psoriasis, and it can be one of the most challenging aspects of the disease. It can be difficult to treat for several reasons. First, hair can interfere with topical therapy reaching its site of action on the scalp. Second, facial skin also can be exposed to these treatments with the associated risk for adverse events. Finally, compliance often is difficult.

An evidence-based review published online on September 21 in the American Journal of Clinical Dermatology examined treatments for scalp psoriasis, including newer systemic therapies. Of 475 studies initially identified from PubMed and 845 from Embase (up to May 2016), the review included 27 clinical trials, 4 papers reporting pooled analyses of other clinical trials, 10 open-label trials, 1 case series, and 2 case reports after excluding non-English literature.

Wang and Tsai noted that few randomized controlled trials have been performed specifically in scalp psoriasis. The authors found that topical corticosteroids provide good effects and are usually recommended as first-line treatment. Calcipotriol–betamethasone dipropionate is more highly effective than either of its individual components.

The analysis also suggested that localized phototherapy is better than generalized phototherapy on hair-bearing areas. Methotrexate, cyclosporine, fumaric acid esters, and acitretin are well-recognized agents in the treatment of psoriasis, but they located no published randomized controlled trials specifically evaluating these agents in scalp psoriasis. Wang and Tsai also commented that biologics and new small-molecule agents show excellent effects on scalp psoriasis, but the high cost of these treatments mean they may be limited to use in extensive scalp psoriasis. They suggested that more controlled studies are needed for an evidence-based approach to scalp psoriasis.

What’s the issue?

Scalp psoriasis can be an isolated condition or may occur in association with more extensive disease. There has been increased attention to its treatment over the last several years, with several new options. What is your preferred approach to scalp psoriasis?

We want to know your views! Tell us what you think.

Scalp psoriasis often is the initial presentation of psoriasis, and it can be one of the most challenging aspects of the disease. It can be difficult to treat for several reasons. First, hair can interfere with topical therapy reaching its site of action on the scalp. Second, facial skin also can be exposed to these treatments with the associated risk for adverse events. Finally, compliance often is difficult.

An evidence-based review published online on September 21 in the American Journal of Clinical Dermatology examined treatments for scalp psoriasis, including newer systemic therapies. Of 475 studies initially identified from PubMed and 845 from Embase (up to May 2016), the review included 27 clinical trials, 4 papers reporting pooled analyses of other clinical trials, 10 open-label trials, 1 case series, and 2 case reports after excluding non-English literature.

Wang and Tsai noted that few randomized controlled trials have been performed specifically in scalp psoriasis. The authors found that topical corticosteroids provide good effects and are usually recommended as first-line treatment. Calcipotriol–betamethasone dipropionate is more highly effective than either of its individual components.

The analysis also suggested that localized phototherapy is better than generalized phototherapy on hair-bearing areas. Methotrexate, cyclosporine, fumaric acid esters, and acitretin are well-recognized agents in the treatment of psoriasis, but they located no published randomized controlled trials specifically evaluating these agents in scalp psoriasis. Wang and Tsai also commented that biologics and new small-molecule agents show excellent effects on scalp psoriasis, but the high cost of these treatments mean they may be limited to use in extensive scalp psoriasis. They suggested that more controlled studies are needed for an evidence-based approach to scalp psoriasis.

What’s the issue?

Scalp psoriasis can be an isolated condition or may occur in association with more extensive disease. There has been increased attention to its treatment over the last several years, with several new options. What is your preferred approach to scalp psoriasis?

We want to know your views! Tell us what you think.

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Biopsy scalp area for alopecia diagnosis

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– Getting a proper scalp biopsy and providing the dermatopathologist with a good supporting history are important elements in diagnosing a patient with hair loss, according to Eleanor Knopp, MD, a dermatologist and dermatopathologist with Group Health Permanente, Seattle.

Dr. Eleanor Knopp
A 4-mm punch biopsy has almost twice the surface area of a 3-mm biopsy (12.6 mm2 compared with about 7 mm2), she pointed out. With the larger biopsy, “the patient is not going to know the difference … and I get so many more follicles to look at as a pathologist,” she said, “so that’s a really easy increased bang for your buck.”

Evaluating for the presence or absence of follicular ostia with a dermatoscope helps distinguish scarring from nonscarring alopecia. Scarring alopecias typically show loss of follicular ostia, she noted.

While this method is effective at identifying nonscarring areas in white patients, it can be difficult to appreciate the disappearance of follicular ostia with a dermatoscope in patients of African descent or patients with darkly pigmented skin. In these patients, eccrine ostia appear as white pinpoint dots under a dermatoscope and mimic the appearance of follicular ostia, despite the presence of scarring alopecia, she noted.

In this situation, Dr. Knopp said the threshold for biopsying patients with darkly pigmented skin should be lower to rule out an early cicatricial alopecia.

For any specimen sent to the dermatopathologist, it is important to note patient characteristics, including age and race, duration of the condition, and clinical pattern. Not only is race helpful for interpreting what is seen in the specimen, but certain racial groups have higher predilections for certain diseases. There are also differences in normal hair densities depending on race although there can be a wide range even within a race, she added. Providing a photo of the involved area of the scalp is also a good idea, she added.

When biopsying a scarring alopecia, Dr. Knopp said that her preference is to find an area of relatively early thinning with visible erythema, and scale if it is present, “so that you know you have active inflammatory disease, but it’s not so advanced that you’re just seeing end-stage changes of scarring.”

It is worth having a discussion with the dermatopathologist about sectioning specimens, Dr. Knopp said. The consensus among most dermatopathologists is that horizontal sections are “absolutely the way to go for nonscarring alopecias,” but some dermatopathologists strongly prefer vertical sections, especially in cicatricial alopecias. Clinicians can always choose among the many reference laboratories to obtain the type of sections they prefer.

In cases of cicatricial alopecia, Dr. Knopp cautioned that clinicians may see “juicy pustules or fluctuant nodules” and consider these findings indicative of a highly active area of disease, but these changes may obscure early findings that are helpful to a pathologist. A better choice for a biopsy site is an area of early involvement that is not too inflamed and not so advanced that it is just scar, she noted.

Another potential pitfall is the temptation to biopsy a tuft of hairs. If there is polytrichia or compounding of follicles, it may be tempting to fit the punch tool over what are also sometimes called “doll’s hairs.” But those structures are nonspecific, end-stage features of many different cicatricial alopecias, including lichen planopilaris, central centrifugal cicatricial alopecia, and even lupus. Instead, Dr. Knopp recommended taking a specimen at the periphery where compounding is not present but where there is thinning and active inflammation.

Dr. Knopp, also with the University of Washington, Seattle, reported no financial relationships.
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– Getting a proper scalp biopsy and providing the dermatopathologist with a good supporting history are important elements in diagnosing a patient with hair loss, according to Eleanor Knopp, MD, a dermatologist and dermatopathologist with Group Health Permanente, Seattle.

Dr. Eleanor Knopp
A 4-mm punch biopsy has almost twice the surface area of a 3-mm biopsy (12.6 mm2 compared with about 7 mm2), she pointed out. With the larger biopsy, “the patient is not going to know the difference … and I get so many more follicles to look at as a pathologist,” she said, “so that’s a really easy increased bang for your buck.”

Evaluating for the presence or absence of follicular ostia with a dermatoscope helps distinguish scarring from nonscarring alopecia. Scarring alopecias typically show loss of follicular ostia, she noted.

While this method is effective at identifying nonscarring areas in white patients, it can be difficult to appreciate the disappearance of follicular ostia with a dermatoscope in patients of African descent or patients with darkly pigmented skin. In these patients, eccrine ostia appear as white pinpoint dots under a dermatoscope and mimic the appearance of follicular ostia, despite the presence of scarring alopecia, she noted.

In this situation, Dr. Knopp said the threshold for biopsying patients with darkly pigmented skin should be lower to rule out an early cicatricial alopecia.

For any specimen sent to the dermatopathologist, it is important to note patient characteristics, including age and race, duration of the condition, and clinical pattern. Not only is race helpful for interpreting what is seen in the specimen, but certain racial groups have higher predilections for certain diseases. There are also differences in normal hair densities depending on race although there can be a wide range even within a race, she added. Providing a photo of the involved area of the scalp is also a good idea, she added.

When biopsying a scarring alopecia, Dr. Knopp said that her preference is to find an area of relatively early thinning with visible erythema, and scale if it is present, “so that you know you have active inflammatory disease, but it’s not so advanced that you’re just seeing end-stage changes of scarring.”

It is worth having a discussion with the dermatopathologist about sectioning specimens, Dr. Knopp said. The consensus among most dermatopathologists is that horizontal sections are “absolutely the way to go for nonscarring alopecias,” but some dermatopathologists strongly prefer vertical sections, especially in cicatricial alopecias. Clinicians can always choose among the many reference laboratories to obtain the type of sections they prefer.

In cases of cicatricial alopecia, Dr. Knopp cautioned that clinicians may see “juicy pustules or fluctuant nodules” and consider these findings indicative of a highly active area of disease, but these changes may obscure early findings that are helpful to a pathologist. A better choice for a biopsy site is an area of early involvement that is not too inflamed and not so advanced that it is just scar, she noted.

Another potential pitfall is the temptation to biopsy a tuft of hairs. If there is polytrichia or compounding of follicles, it may be tempting to fit the punch tool over what are also sometimes called “doll’s hairs.” But those structures are nonspecific, end-stage features of many different cicatricial alopecias, including lichen planopilaris, central centrifugal cicatricial alopecia, and even lupus. Instead, Dr. Knopp recommended taking a specimen at the periphery where compounding is not present but where there is thinning and active inflammation.

Dr. Knopp, also with the University of Washington, Seattle, reported no financial relationships.

 

– Getting a proper scalp biopsy and providing the dermatopathologist with a good supporting history are important elements in diagnosing a patient with hair loss, according to Eleanor Knopp, MD, a dermatologist and dermatopathologist with Group Health Permanente, Seattle.

Dr. Eleanor Knopp
A 4-mm punch biopsy has almost twice the surface area of a 3-mm biopsy (12.6 mm2 compared with about 7 mm2), she pointed out. With the larger biopsy, “the patient is not going to know the difference … and I get so many more follicles to look at as a pathologist,” she said, “so that’s a really easy increased bang for your buck.”

Evaluating for the presence or absence of follicular ostia with a dermatoscope helps distinguish scarring from nonscarring alopecia. Scarring alopecias typically show loss of follicular ostia, she noted.

While this method is effective at identifying nonscarring areas in white patients, it can be difficult to appreciate the disappearance of follicular ostia with a dermatoscope in patients of African descent or patients with darkly pigmented skin. In these patients, eccrine ostia appear as white pinpoint dots under a dermatoscope and mimic the appearance of follicular ostia, despite the presence of scarring alopecia, she noted.

In this situation, Dr. Knopp said the threshold for biopsying patients with darkly pigmented skin should be lower to rule out an early cicatricial alopecia.

For any specimen sent to the dermatopathologist, it is important to note patient characteristics, including age and race, duration of the condition, and clinical pattern. Not only is race helpful for interpreting what is seen in the specimen, but certain racial groups have higher predilections for certain diseases. There are also differences in normal hair densities depending on race although there can be a wide range even within a race, she added. Providing a photo of the involved area of the scalp is also a good idea, she added.

When biopsying a scarring alopecia, Dr. Knopp said that her preference is to find an area of relatively early thinning with visible erythema, and scale if it is present, “so that you know you have active inflammatory disease, but it’s not so advanced that you’re just seeing end-stage changes of scarring.”

It is worth having a discussion with the dermatopathologist about sectioning specimens, Dr. Knopp said. The consensus among most dermatopathologists is that horizontal sections are “absolutely the way to go for nonscarring alopecias,” but some dermatopathologists strongly prefer vertical sections, especially in cicatricial alopecias. Clinicians can always choose among the many reference laboratories to obtain the type of sections they prefer.

In cases of cicatricial alopecia, Dr. Knopp cautioned that clinicians may see “juicy pustules or fluctuant nodules” and consider these findings indicative of a highly active area of disease, but these changes may obscure early findings that are helpful to a pathologist. A better choice for a biopsy site is an area of early involvement that is not too inflamed and not so advanced that it is just scar, she noted.

Another potential pitfall is the temptation to biopsy a tuft of hairs. If there is polytrichia or compounding of follicles, it may be tempting to fit the punch tool over what are also sometimes called “doll’s hairs.” But those structures are nonspecific, end-stage features of many different cicatricial alopecias, including lichen planopilaris, central centrifugal cicatricial alopecia, and even lupus. Instead, Dr. Knopp recommended taking a specimen at the periphery where compounding is not present but where there is thinning and active inflammation.

Dr. Knopp, also with the University of Washington, Seattle, reported no financial relationships.
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Cosmetic Corner: Dermatologists Weigh in on Nail Care Products

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To improve patient care and outcomes, leading dermatologists offered their recommendations on nail care products. Consideration must be given to:

  • Aquaphor Healing Ointment
    Beiersdorf, Inc

    Recommended by Gary Goldenberg, MD, New York, New York

 

  • Biotin Oral Supplements
    Manufacturers vary

    “Biotin is a helpful supplement for brittle nails. It may take 6 months to see improvement in the nails.”—Shari Lipner, MD, PhD, New York, New York

    Recommended by Gary Goldenberg, MD, New York, New York

 

  • Deep Comfort Hand and Cuticle Cream
    Clinique

    “It has good hydration for cuticles with sodium hyaluronate and squalene. It also is fragrance free.”—Anthony M. Rossi, MD, New York, New York

 

  • Genadur
    Medimetriks Pharmaceuticals, Inc

    Recommended by Gary Goldenberg, MD, New York, New York

 

  • Lanolin-Rich Nail Conditioner
    Elon

    “It’s great for moisturizing and nail hardening.”—Marta Rendon, MD, Boca Raton, Florida

 

  • Nail Renewal System
    Dr. Dana

    “Developed by dermatologist Dr. Dana Stern, the system combines glycolic acid to improve discoloration and ridging, along with hydrating and strengthening botanicals to improve the look, feel, and overall health of the nails.”— Joshua Zeichner, MD, New York, New York

 

Cutis invites readers to send us their recommendations. Acne scar treatments, self-tanners, and cleansing devices will be featured in upcoming editions of Cosmetic Corner. Please e-mail your recommendation(s) to the Editorial Office.

Disclaimer: Opinions expressed herein do not necessarily reflect those of Cutis or Frontline Medical Communications Inc. and shall not be used for product endorsement purposes. Any reference made to a specific commercial product does not indicate or imply that Cutis or Frontline Medical Communications Inc. endorses, recommends, or favors the product mentioned. No guarantee is given to the effects of recommended products.

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To improve patient care and outcomes, leading dermatologists offered their recommendations on nail care products. Consideration must be given to:

  • Aquaphor Healing Ointment
    Beiersdorf, Inc

    Recommended by Gary Goldenberg, MD, New York, New York

 

  • Biotin Oral Supplements
    Manufacturers vary

    “Biotin is a helpful supplement for brittle nails. It may take 6 months to see improvement in the nails.”—Shari Lipner, MD, PhD, New York, New York

    Recommended by Gary Goldenberg, MD, New York, New York

 

  • Deep Comfort Hand and Cuticle Cream
    Clinique

    “It has good hydration for cuticles with sodium hyaluronate and squalene. It also is fragrance free.”—Anthony M. Rossi, MD, New York, New York

 

  • Genadur
    Medimetriks Pharmaceuticals, Inc

    Recommended by Gary Goldenberg, MD, New York, New York

 

  • Lanolin-Rich Nail Conditioner
    Elon

    “It’s great for moisturizing and nail hardening.”—Marta Rendon, MD, Boca Raton, Florida

 

  • Nail Renewal System
    Dr. Dana

    “Developed by dermatologist Dr. Dana Stern, the system combines glycolic acid to improve discoloration and ridging, along with hydrating and strengthening botanicals to improve the look, feel, and overall health of the nails.”— Joshua Zeichner, MD, New York, New York

 

Cutis invites readers to send us their recommendations. Acne scar treatments, self-tanners, and cleansing devices will be featured in upcoming editions of Cosmetic Corner. Please e-mail your recommendation(s) to the Editorial Office.

Disclaimer: Opinions expressed herein do not necessarily reflect those of Cutis or Frontline Medical Communications Inc. and shall not be used for product endorsement purposes. Any reference made to a specific commercial product does not indicate or imply that Cutis or Frontline Medical Communications Inc. endorses, recommends, or favors the product mentioned. No guarantee is given to the effects of recommended products.

To improve patient care and outcomes, leading dermatologists offered their recommendations on nail care products. Consideration must be given to:

  • Aquaphor Healing Ointment
    Beiersdorf, Inc

    Recommended by Gary Goldenberg, MD, New York, New York

 

  • Biotin Oral Supplements
    Manufacturers vary

    “Biotin is a helpful supplement for brittle nails. It may take 6 months to see improvement in the nails.”—Shari Lipner, MD, PhD, New York, New York

    Recommended by Gary Goldenberg, MD, New York, New York

 

  • Deep Comfort Hand and Cuticle Cream
    Clinique

    “It has good hydration for cuticles with sodium hyaluronate and squalene. It also is fragrance free.”—Anthony M. Rossi, MD, New York, New York

 

  • Genadur
    Medimetriks Pharmaceuticals, Inc

    Recommended by Gary Goldenberg, MD, New York, New York

 

  • Lanolin-Rich Nail Conditioner
    Elon

    “It’s great for moisturizing and nail hardening.”—Marta Rendon, MD, Boca Raton, Florida

 

  • Nail Renewal System
    Dr. Dana

    “Developed by dermatologist Dr. Dana Stern, the system combines glycolic acid to improve discoloration and ridging, along with hydrating and strengthening botanicals to improve the look, feel, and overall health of the nails.”— Joshua Zeichner, MD, New York, New York

 

Cutis invites readers to send us their recommendations. Acne scar treatments, self-tanners, and cleansing devices will be featured in upcoming editions of Cosmetic Corner. Please e-mail your recommendation(s) to the Editorial Office.

Disclaimer: Opinions expressed herein do not necessarily reflect those of Cutis or Frontline Medical Communications Inc. and shall not be used for product endorsement purposes. Any reference made to a specific commercial product does not indicate or imply that Cutis or Frontline Medical Communications Inc. endorses, recommends, or favors the product mentioned. No guarantee is given to the effects of recommended products.

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An Unusual Case of Folliculitis Spinulosa Decalvans

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Case Report

A 24-year-old man was referred to the dermatology department for evaluation of pustules, atrophic scars, and alopecia on the scalp of 6 years’ duration. Six years prior, erythema, scaling, and follicular keratotic papules had appeared on the superciliary arches, and he started to lose hair from the eyebrows. Three months later, he developed mildly pruritic and painful scaling and pustules on the scalp. These lesions resolved with atrophic scarring accompanied by alopecia. One year later, follicular keratotic papules developed on the cheeks, chest, abdomen, back, lateral upper arms, thighs, and axillae. Two years later, direct microscopy of the lesions on the scalp and fungal culture were negative. After 2 weeks of treatment with roxithromycin (0.15 g twice daily), the scalp pustules dried out and resolved; however, they recurred when the patient stopped taking the medication. Six months later, he was started on isotretinoin treatment (10 mg once daily) for half a year, but no improvement was seen. His parents were nonconsanguineous, and no other family members were affected.

Dermatologic examination revealed large areas of atrophic scarring and alopecia on the scalp. Only a few solitary hairs remained on the top of the head, with the follicles surrounded by keratotic papules, pustules, and black scabs. There was sparse hair on the forehead and temples and scattered hair clusters in the occipital region near the hairline. These follicles also were associated with keratotic papules (Figure 1A). Erythema, scales, and follicular keratotic papules of the superciliary arches with sparse eyebrows and axillary hairs were noted. Follicular keratotic papules also were observed on the cheeks, axillae, chest, abdomen, back, lateral upper arms, and thighs. Dental examination revealed a large space between the upper anterior teeth and the lower anterior teeth. The upper anterior teeth were anteverted, there was congenital absence of right lower central incisors, and the anterior teeth were in deep overbite and overjet (Figure 1B). There was gingival atrophy and calculus dentalis in the upper and lower teeth. He had a fissured tongue with atrophic filiform papillae (Figure 1C).

Figure 1. Characteristic lesions of folliculitis spinulosa decalvans on the scalp (A). This patient also presented with a large space between the upper anterior teeth and lower anterior teeth (B). Anteversion of the upper anterior teeth, congenital absence of right lower central incisors, and deep overbite and overjet of the anterior teeth also were seen. A fissured tongue with atrophic filiform papillae also was present (C).

Figure 2. A panoramic radiograph of the occlusal surface revealed congenital absence of right lower central incisors.

Laboratory testing of the blood, urine, stool, hepatic and renal function, and serum vitamin B2 and B12 levelswere all within reference range. A panoramic radiograph of the occlusal surface showed congenital absence of right lower central incisors (Figure 2), and a lateral projection of a cranial radiograph confirmed that the anterior teeth were in deep overbite and overjet. Direct microscopy and fungal culture of material collected from the dorsal tongue were negative. Direct microscopy and fungal culture of diseased hairs also were negative. A rapid plasma reagin test, Treponema pallidum hemagglutination assay, and human immunodeficiency virus test were negative. Staphylococcus aureus was isolated from the scalp pustules, and in vitro drug susceptibility testing showed that it was sensitive to clarithromycin and moxifloxacin. Pathological examination of a biopsy of the occipital skin lesions showed a thickened epidermal spinous layer and massive infiltration of plasma cells, neutrophils, and multinucleated giant cells around the hair follicles (Figure 3). Pathological examination of the skin lesions on the superciliary arch also showed infiltration of inflammatory cells in the dermis around the hair follicles.
Figure 3. Photomicrographs showed parakeratosis; acanthosis; and massive infiltration of inflammatory cells, mainly plasma cells and neutrophils, around the hair follicles (A and B)(H&E, original magnifications ×100 and ×400).

Based on these findings, a diagnosis of folliculitis spinulosa decalvans (FSD) was made and the patient was started on clarithromycin (0.25 g twice daily), metronidazole (0.2 g 3 times daily), viaminate (50 mg 3 times daily), and fusidic acid cream (coating the affected area twice daily). When he returned for follow-up 1 month later, the pustules had disappeared and the black scabs had fallen off, leaving atrophic scars. The long-term efficacy of this regimen is still under observation.

Comment

Folliculitis spinulosa decalvans, along with keratosis follicularis spinulosa decalvans (KFSD), keratosis pilaris atrophicans faciei, and atrophoderma vermiculatum, belongs to a group of diseases that includes keratosis pilaris atrophicans. In 1994, Oranje et al1 suggested the term folliculitis spinulosa decalvans, with signs including persistent pustules, characteristic keratotic papules, and scarring alopecia of the scalp, which may be exacerbated at puberty. Staphylococcus aureus was isolated from the pustules in one study2; however, in another study, repeated cultures were negative.3 Although the main inheritance pattern of KFSD is X-linked, autosomal-dominant inheritance is more common in FSD. Furthermore, there are certain differences in the clinical manifestations of these 2 conditions. Therefore, it remains controversial if FSD is an independent disease or merely a subtype of KFSD.

 

 

Our patient’s symptoms manifested after puberty, primarily pustules as well as atrophic and scarring alopecia of the scalp and follicular keratotic papules on the head, face, trunk, lateral upper arms, and thighs. Pathologic examination showed massive infiltration of plasma cells, neutrophils, and multinucleated giant cells around the hair follicles. The clinical and histopathologic findings met the diagnostic criteria for FSD.

Folliculitis spinulosa decalvans is a rare clinical condition with few cases reported.3-5 In addition to the aforementioned characteristic clinical manifestations, our patient also had dental anomalies, a fissured tongue, and atrophy of the tongue papillae, which are not known to be associated with FSD. Dental anomalies are characteristic of patients with Down syndrome, ectodermal dysplasia, Papillon-Lefèvre syndrome, and other conditions.6 Fissured tongue is a normal variant that occurs in 5% to 11% of individuals. It also is a classic but nonspecific feature of Melkersson-Rosenthal syndrome and may occur in psoriasis, Down syndrome, acromegaly, and Sjögren syndrome.7 Atrophy of the tongue papillae is associated with anemia, pellagra, Sjögren syndrome, candidiasis, and other conditions.8 Because there are no known reports of associations between FSD and any of these oral manifestations, it is possible that they were unrelated to FSD in our patient.

Folliculitis spinulosa decalvans usually is recurrent and there is no consistently effective treatment for it. Kunte et al4 reported that dapsone (100 mg/d) led to resolution of scalp inflammation and pustules within 1 month. Romine et al2 reported that a 3-week course of dichloroxacillin (250 mg 4 times daily) induced disappearance of pustules around the hair follicles. However, Hallai et al5 reported a patient who was resistant to isotretinoin treatment. In our case, after 1 month of treatment with clarithromycin, metronidazole, viaminate, and fusidic acid cream, the pustules had resolved and the black scabs had fallen off, leaving atrophic scars. The long-term efficacy of this regimen is still under observation.

Conclusion

We report a case of FSD with dental anomalies, a fissured tongue, and atrophy of tongue papillae, none of which have previously been reported in association with FSD. We, therefore, believe that our patient’s oral manifestations are unrelated to FSD.

References
  1. Oranje AP, van Osch LD, Oosterwijk JC. Keratosis pilaris atrophicans. one heterogeneous disease or a symptom in different clinical entities? Arch Dermatol. 1994;13:500-502.
  2. Romine KA, Rothschild JG, Hansen RC. Cicatricial alopecia and keratosis pilaris. keratosis follicularis spinulosa decalvans. Arch Dermatol. 1997;13:381-384.
  3. Di Lernia V, Ricci C. Folliculitis spinulosa decalvans: an uncommon entity within the keratosis pilaris atrophicans spectrum. Pediatr Dermatol. 2006;23:255-258.
  4. Kunte C, Loeser C, Wolff H. Folliculitis spinulosa decalvans: successful therapy with dapsone. J Am Acad Dermatol. 1998;39(5, pt 2):891-892.
  5. Hallai N, Thompson I, Williams P, et al. Folliculitis spinulosa decalvans: failure to respond to oral isotretinoin. J Eur Acad Dermatol Venereol. 2006;20:223-224.
  6. Scully C, Hegarty A. The oral cavity and lips. In: Burns T, Breathnach S, Cox N, et al. Rook’s Textbook of Dermatology. 8th ed. Oxford, England: Wiley-Blackwell; 2010:69.7-69.10.
  7. Wolff K, Goldsmith LA, Katz SI, et al. Fitzpatrick’s Dermatology in General Medicine. 7th ed. New York, NY: McGraw-Hill Companies; 2007:643.
  8. Mulliken RA, Casner MJ. Oral manifestations of systemic disease. Emerg Med Clin North Am. 2000;18:565-575.
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Dr. Fan is from the Department of Dermatology, First People’s Hospital of Changshu City, Changshu Hospital Affiliated to Soochow University, Changshu, Jiangsu Province, People’s Republic of China. Dr. Zhang is from the Department of Dermatology, Traditional Chinese Medical Hospital of Changshu City. Dr. Song is from the Department of Dermatology, The First Hospital Affiliated to Soochow University, Suzhou, Jiangsu Province.

The authors report no conflict of interest.

Correspondence: Wenge Fan, MD, Department of Dermatology, First People’s Hospital of Changshu City, Changshu Hospital Affiliated to Soochow University, Changshu 215500, Jiangsu Province, People’s Republic of China ([email protected]).

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Dr. Fan is from the Department of Dermatology, First People’s Hospital of Changshu City, Changshu Hospital Affiliated to Soochow University, Changshu, Jiangsu Province, People’s Republic of China. Dr. Zhang is from the Department of Dermatology, Traditional Chinese Medical Hospital of Changshu City. Dr. Song is from the Department of Dermatology, The First Hospital Affiliated to Soochow University, Suzhou, Jiangsu Province.

The authors report no conflict of interest.

Correspondence: Wenge Fan, MD, Department of Dermatology, First People’s Hospital of Changshu City, Changshu Hospital Affiliated to Soochow University, Changshu 215500, Jiangsu Province, People’s Republic of China ([email protected]).

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Dr. Fan is from the Department of Dermatology, First People’s Hospital of Changshu City, Changshu Hospital Affiliated to Soochow University, Changshu, Jiangsu Province, People’s Republic of China. Dr. Zhang is from the Department of Dermatology, Traditional Chinese Medical Hospital of Changshu City. Dr. Song is from the Department of Dermatology, The First Hospital Affiliated to Soochow University, Suzhou, Jiangsu Province.

The authors report no conflict of interest.

Correspondence: Wenge Fan, MD, Department of Dermatology, First People’s Hospital of Changshu City, Changshu Hospital Affiliated to Soochow University, Changshu 215500, Jiangsu Province, People’s Republic of China ([email protected]).

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Case Report

A 24-year-old man was referred to the dermatology department for evaluation of pustules, atrophic scars, and alopecia on the scalp of 6 years’ duration. Six years prior, erythema, scaling, and follicular keratotic papules had appeared on the superciliary arches, and he started to lose hair from the eyebrows. Three months later, he developed mildly pruritic and painful scaling and pustules on the scalp. These lesions resolved with atrophic scarring accompanied by alopecia. One year later, follicular keratotic papules developed on the cheeks, chest, abdomen, back, lateral upper arms, thighs, and axillae. Two years later, direct microscopy of the lesions on the scalp and fungal culture were negative. After 2 weeks of treatment with roxithromycin (0.15 g twice daily), the scalp pustules dried out and resolved; however, they recurred when the patient stopped taking the medication. Six months later, he was started on isotretinoin treatment (10 mg once daily) for half a year, but no improvement was seen. His parents were nonconsanguineous, and no other family members were affected.

Dermatologic examination revealed large areas of atrophic scarring and alopecia on the scalp. Only a few solitary hairs remained on the top of the head, with the follicles surrounded by keratotic papules, pustules, and black scabs. There was sparse hair on the forehead and temples and scattered hair clusters in the occipital region near the hairline. These follicles also were associated with keratotic papules (Figure 1A). Erythema, scales, and follicular keratotic papules of the superciliary arches with sparse eyebrows and axillary hairs were noted. Follicular keratotic papules also were observed on the cheeks, axillae, chest, abdomen, back, lateral upper arms, and thighs. Dental examination revealed a large space between the upper anterior teeth and the lower anterior teeth. The upper anterior teeth were anteverted, there was congenital absence of right lower central incisors, and the anterior teeth were in deep overbite and overjet (Figure 1B). There was gingival atrophy and calculus dentalis in the upper and lower teeth. He had a fissured tongue with atrophic filiform papillae (Figure 1C).

Figure 1. Characteristic lesions of folliculitis spinulosa decalvans on the scalp (A). This patient also presented with a large space between the upper anterior teeth and lower anterior teeth (B). Anteversion of the upper anterior teeth, congenital absence of right lower central incisors, and deep overbite and overjet of the anterior teeth also were seen. A fissured tongue with atrophic filiform papillae also was present (C).

Figure 2. A panoramic radiograph of the occlusal surface revealed congenital absence of right lower central incisors.

Laboratory testing of the blood, urine, stool, hepatic and renal function, and serum vitamin B2 and B12 levelswere all within reference range. A panoramic radiograph of the occlusal surface showed congenital absence of right lower central incisors (Figure 2), and a lateral projection of a cranial radiograph confirmed that the anterior teeth were in deep overbite and overjet. Direct microscopy and fungal culture of material collected from the dorsal tongue were negative. Direct microscopy and fungal culture of diseased hairs also were negative. A rapid plasma reagin test, Treponema pallidum hemagglutination assay, and human immunodeficiency virus test were negative. Staphylococcus aureus was isolated from the scalp pustules, and in vitro drug susceptibility testing showed that it was sensitive to clarithromycin and moxifloxacin. Pathological examination of a biopsy of the occipital skin lesions showed a thickened epidermal spinous layer and massive infiltration of plasma cells, neutrophils, and multinucleated giant cells around the hair follicles (Figure 3). Pathological examination of the skin lesions on the superciliary arch also showed infiltration of inflammatory cells in the dermis around the hair follicles.
Figure 3. Photomicrographs showed parakeratosis; acanthosis; and massive infiltration of inflammatory cells, mainly plasma cells and neutrophils, around the hair follicles (A and B)(H&E, original magnifications ×100 and ×400).

Based on these findings, a diagnosis of folliculitis spinulosa decalvans (FSD) was made and the patient was started on clarithromycin (0.25 g twice daily), metronidazole (0.2 g 3 times daily), viaminate (50 mg 3 times daily), and fusidic acid cream (coating the affected area twice daily). When he returned for follow-up 1 month later, the pustules had disappeared and the black scabs had fallen off, leaving atrophic scars. The long-term efficacy of this regimen is still under observation.

Comment

Folliculitis spinulosa decalvans, along with keratosis follicularis spinulosa decalvans (KFSD), keratosis pilaris atrophicans faciei, and atrophoderma vermiculatum, belongs to a group of diseases that includes keratosis pilaris atrophicans. In 1994, Oranje et al1 suggested the term folliculitis spinulosa decalvans, with signs including persistent pustules, characteristic keratotic papules, and scarring alopecia of the scalp, which may be exacerbated at puberty. Staphylococcus aureus was isolated from the pustules in one study2; however, in another study, repeated cultures were negative.3 Although the main inheritance pattern of KFSD is X-linked, autosomal-dominant inheritance is more common in FSD. Furthermore, there are certain differences in the clinical manifestations of these 2 conditions. Therefore, it remains controversial if FSD is an independent disease or merely a subtype of KFSD.

 

 

Our patient’s symptoms manifested after puberty, primarily pustules as well as atrophic and scarring alopecia of the scalp and follicular keratotic papules on the head, face, trunk, lateral upper arms, and thighs. Pathologic examination showed massive infiltration of plasma cells, neutrophils, and multinucleated giant cells around the hair follicles. The clinical and histopathologic findings met the diagnostic criteria for FSD.

Folliculitis spinulosa decalvans is a rare clinical condition with few cases reported.3-5 In addition to the aforementioned characteristic clinical manifestations, our patient also had dental anomalies, a fissured tongue, and atrophy of the tongue papillae, which are not known to be associated with FSD. Dental anomalies are characteristic of patients with Down syndrome, ectodermal dysplasia, Papillon-Lefèvre syndrome, and other conditions.6 Fissured tongue is a normal variant that occurs in 5% to 11% of individuals. It also is a classic but nonspecific feature of Melkersson-Rosenthal syndrome and may occur in psoriasis, Down syndrome, acromegaly, and Sjögren syndrome.7 Atrophy of the tongue papillae is associated with anemia, pellagra, Sjögren syndrome, candidiasis, and other conditions.8 Because there are no known reports of associations between FSD and any of these oral manifestations, it is possible that they were unrelated to FSD in our patient.

Folliculitis spinulosa decalvans usually is recurrent and there is no consistently effective treatment for it. Kunte et al4 reported that dapsone (100 mg/d) led to resolution of scalp inflammation and pustules within 1 month. Romine et al2 reported that a 3-week course of dichloroxacillin (250 mg 4 times daily) induced disappearance of pustules around the hair follicles. However, Hallai et al5 reported a patient who was resistant to isotretinoin treatment. In our case, after 1 month of treatment with clarithromycin, metronidazole, viaminate, and fusidic acid cream, the pustules had resolved and the black scabs had fallen off, leaving atrophic scars. The long-term efficacy of this regimen is still under observation.

Conclusion

We report a case of FSD with dental anomalies, a fissured tongue, and atrophy of tongue papillae, none of which have previously been reported in association with FSD. We, therefore, believe that our patient’s oral manifestations are unrelated to FSD.

Case Report

A 24-year-old man was referred to the dermatology department for evaluation of pustules, atrophic scars, and alopecia on the scalp of 6 years’ duration. Six years prior, erythema, scaling, and follicular keratotic papules had appeared on the superciliary arches, and he started to lose hair from the eyebrows. Three months later, he developed mildly pruritic and painful scaling and pustules on the scalp. These lesions resolved with atrophic scarring accompanied by alopecia. One year later, follicular keratotic papules developed on the cheeks, chest, abdomen, back, lateral upper arms, thighs, and axillae. Two years later, direct microscopy of the lesions on the scalp and fungal culture were negative. After 2 weeks of treatment with roxithromycin (0.15 g twice daily), the scalp pustules dried out and resolved; however, they recurred when the patient stopped taking the medication. Six months later, he was started on isotretinoin treatment (10 mg once daily) for half a year, but no improvement was seen. His parents were nonconsanguineous, and no other family members were affected.

Dermatologic examination revealed large areas of atrophic scarring and alopecia on the scalp. Only a few solitary hairs remained on the top of the head, with the follicles surrounded by keratotic papules, pustules, and black scabs. There was sparse hair on the forehead and temples and scattered hair clusters in the occipital region near the hairline. These follicles also were associated with keratotic papules (Figure 1A). Erythema, scales, and follicular keratotic papules of the superciliary arches with sparse eyebrows and axillary hairs were noted. Follicular keratotic papules also were observed on the cheeks, axillae, chest, abdomen, back, lateral upper arms, and thighs. Dental examination revealed a large space between the upper anterior teeth and the lower anterior teeth. The upper anterior teeth were anteverted, there was congenital absence of right lower central incisors, and the anterior teeth were in deep overbite and overjet (Figure 1B). There was gingival atrophy and calculus dentalis in the upper and lower teeth. He had a fissured tongue with atrophic filiform papillae (Figure 1C).

Figure 1. Characteristic lesions of folliculitis spinulosa decalvans on the scalp (A). This patient also presented with a large space between the upper anterior teeth and lower anterior teeth (B). Anteversion of the upper anterior teeth, congenital absence of right lower central incisors, and deep overbite and overjet of the anterior teeth also were seen. A fissured tongue with atrophic filiform papillae also was present (C).

Figure 2. A panoramic radiograph of the occlusal surface revealed congenital absence of right lower central incisors.

Laboratory testing of the blood, urine, stool, hepatic and renal function, and serum vitamin B2 and B12 levelswere all within reference range. A panoramic radiograph of the occlusal surface showed congenital absence of right lower central incisors (Figure 2), and a lateral projection of a cranial radiograph confirmed that the anterior teeth were in deep overbite and overjet. Direct microscopy and fungal culture of material collected from the dorsal tongue were negative. Direct microscopy and fungal culture of diseased hairs also were negative. A rapid plasma reagin test, Treponema pallidum hemagglutination assay, and human immunodeficiency virus test were negative. Staphylococcus aureus was isolated from the scalp pustules, and in vitro drug susceptibility testing showed that it was sensitive to clarithromycin and moxifloxacin. Pathological examination of a biopsy of the occipital skin lesions showed a thickened epidermal spinous layer and massive infiltration of plasma cells, neutrophils, and multinucleated giant cells around the hair follicles (Figure 3). Pathological examination of the skin lesions on the superciliary arch also showed infiltration of inflammatory cells in the dermis around the hair follicles.
Figure 3. Photomicrographs showed parakeratosis; acanthosis; and massive infiltration of inflammatory cells, mainly plasma cells and neutrophils, around the hair follicles (A and B)(H&E, original magnifications ×100 and ×400).

Based on these findings, a diagnosis of folliculitis spinulosa decalvans (FSD) was made and the patient was started on clarithromycin (0.25 g twice daily), metronidazole (0.2 g 3 times daily), viaminate (50 mg 3 times daily), and fusidic acid cream (coating the affected area twice daily). When he returned for follow-up 1 month later, the pustules had disappeared and the black scabs had fallen off, leaving atrophic scars. The long-term efficacy of this regimen is still under observation.

Comment

Folliculitis spinulosa decalvans, along with keratosis follicularis spinulosa decalvans (KFSD), keratosis pilaris atrophicans faciei, and atrophoderma vermiculatum, belongs to a group of diseases that includes keratosis pilaris atrophicans. In 1994, Oranje et al1 suggested the term folliculitis spinulosa decalvans, with signs including persistent pustules, characteristic keratotic papules, and scarring alopecia of the scalp, which may be exacerbated at puberty. Staphylococcus aureus was isolated from the pustules in one study2; however, in another study, repeated cultures were negative.3 Although the main inheritance pattern of KFSD is X-linked, autosomal-dominant inheritance is more common in FSD. Furthermore, there are certain differences in the clinical manifestations of these 2 conditions. Therefore, it remains controversial if FSD is an independent disease or merely a subtype of KFSD.

 

 

Our patient’s symptoms manifested after puberty, primarily pustules as well as atrophic and scarring alopecia of the scalp and follicular keratotic papules on the head, face, trunk, lateral upper arms, and thighs. Pathologic examination showed massive infiltration of plasma cells, neutrophils, and multinucleated giant cells around the hair follicles. The clinical and histopathologic findings met the diagnostic criteria for FSD.

Folliculitis spinulosa decalvans is a rare clinical condition with few cases reported.3-5 In addition to the aforementioned characteristic clinical manifestations, our patient also had dental anomalies, a fissured tongue, and atrophy of the tongue papillae, which are not known to be associated with FSD. Dental anomalies are characteristic of patients with Down syndrome, ectodermal dysplasia, Papillon-Lefèvre syndrome, and other conditions.6 Fissured tongue is a normal variant that occurs in 5% to 11% of individuals. It also is a classic but nonspecific feature of Melkersson-Rosenthal syndrome and may occur in psoriasis, Down syndrome, acromegaly, and Sjögren syndrome.7 Atrophy of the tongue papillae is associated with anemia, pellagra, Sjögren syndrome, candidiasis, and other conditions.8 Because there are no known reports of associations between FSD and any of these oral manifestations, it is possible that they were unrelated to FSD in our patient.

Folliculitis spinulosa decalvans usually is recurrent and there is no consistently effective treatment for it. Kunte et al4 reported that dapsone (100 mg/d) led to resolution of scalp inflammation and pustules within 1 month. Romine et al2 reported that a 3-week course of dichloroxacillin (250 mg 4 times daily) induced disappearance of pustules around the hair follicles. However, Hallai et al5 reported a patient who was resistant to isotretinoin treatment. In our case, after 1 month of treatment with clarithromycin, metronidazole, viaminate, and fusidic acid cream, the pustules had resolved and the black scabs had fallen off, leaving atrophic scars. The long-term efficacy of this regimen is still under observation.

Conclusion

We report a case of FSD with dental anomalies, a fissured tongue, and atrophy of tongue papillae, none of which have previously been reported in association with FSD. We, therefore, believe that our patient’s oral manifestations are unrelated to FSD.

References
  1. Oranje AP, van Osch LD, Oosterwijk JC. Keratosis pilaris atrophicans. one heterogeneous disease or a symptom in different clinical entities? Arch Dermatol. 1994;13:500-502.
  2. Romine KA, Rothschild JG, Hansen RC. Cicatricial alopecia and keratosis pilaris. keratosis follicularis spinulosa decalvans. Arch Dermatol. 1997;13:381-384.
  3. Di Lernia V, Ricci C. Folliculitis spinulosa decalvans: an uncommon entity within the keratosis pilaris atrophicans spectrum. Pediatr Dermatol. 2006;23:255-258.
  4. Kunte C, Loeser C, Wolff H. Folliculitis spinulosa decalvans: successful therapy with dapsone. J Am Acad Dermatol. 1998;39(5, pt 2):891-892.
  5. Hallai N, Thompson I, Williams P, et al. Folliculitis spinulosa decalvans: failure to respond to oral isotretinoin. J Eur Acad Dermatol Venereol. 2006;20:223-224.
  6. Scully C, Hegarty A. The oral cavity and lips. In: Burns T, Breathnach S, Cox N, et al. Rook’s Textbook of Dermatology. 8th ed. Oxford, England: Wiley-Blackwell; 2010:69.7-69.10.
  7. Wolff K, Goldsmith LA, Katz SI, et al. Fitzpatrick’s Dermatology in General Medicine. 7th ed. New York, NY: McGraw-Hill Companies; 2007:643.
  8. Mulliken RA, Casner MJ. Oral manifestations of systemic disease. Emerg Med Clin North Am. 2000;18:565-575.
References
  1. Oranje AP, van Osch LD, Oosterwijk JC. Keratosis pilaris atrophicans. one heterogeneous disease or a symptom in different clinical entities? Arch Dermatol. 1994;13:500-502.
  2. Romine KA, Rothschild JG, Hansen RC. Cicatricial alopecia and keratosis pilaris. keratosis follicularis spinulosa decalvans. Arch Dermatol. 1997;13:381-384.
  3. Di Lernia V, Ricci C. Folliculitis spinulosa decalvans: an uncommon entity within the keratosis pilaris atrophicans spectrum. Pediatr Dermatol. 2006;23:255-258.
  4. Kunte C, Loeser C, Wolff H. Folliculitis spinulosa decalvans: successful therapy with dapsone. J Am Acad Dermatol. 1998;39(5, pt 2):891-892.
  5. Hallai N, Thompson I, Williams P, et al. Folliculitis spinulosa decalvans: failure to respond to oral isotretinoin. J Eur Acad Dermatol Venereol. 2006;20:223-224.
  6. Scully C, Hegarty A. The oral cavity and lips. In: Burns T, Breathnach S, Cox N, et al. Rook’s Textbook of Dermatology. 8th ed. Oxford, England: Wiley-Blackwell; 2010:69.7-69.10.
  7. Wolff K, Goldsmith LA, Katz SI, et al. Fitzpatrick’s Dermatology in General Medicine. 7th ed. New York, NY: McGraw-Hill Companies; 2007:643.
  8. Mulliken RA, Casner MJ. Oral manifestations of systemic disease. Emerg Med Clin North Am. 2000;18:565-575.
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  • Folliculitis spinulosa decalvans (FSD) presents with persistent pustules, characteristic keratotic papules, and scarring alopecia of the scalp.
  • In the case described here, oral manifestations also were present but are not characteristic of FSD.
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Subungual Onycholemmal Cyst of the Toenail Mimicking Subungual Melanoma

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Subungual Onycholemmal Cyst of the Toenail Mimicking Subungual Melanoma

Case Report

A 23-year-old woman presented with a horizontal split along the midline of the right great toenail associated with some tenderness of 2 to 3 months’ duration. Approximately 5 years prior, she noticed a bluish-colored area under the nail that had been steadily increasing in size. She denied a history of trauma, drainage, or bleeding. There was no history of other nail abnormalities. Her medications and personal, family, and social history were noncontributory.

Physical examination of the right great toenail revealed a horizontal split of the nail plate with a bluish hue visible under the nail plate (Figure 1A). The remaining toenails and fingernails were normal. A punch biopsy of the nail bed was performed with a presumptive clinical diagnosis of subungual melanoma versus melanocytic nevus versus cyst (Figure 1B). Nail plate avulsion revealed a blackened nail bed dotted with areas of bluish color and a red friable nodule present focally. Upon further inspection, extension was apparent into the distal matrix.

Figure 1. Subungual pressure-induced onycholysis overlying bluish discoloration of the lateral third of the right great toenail. The proximal and lateral nail folds were unaffected (A). A 4-mm punch biopsy site was visible on the nail bed following partial nail avulsion (B).

Histopathologic examination revealed a cystic structure with an epithelial lining mostly reminiscent of an isthmus catagen cyst admixed with the presence of both an intermittent focal granular layer and an eosinophilic cuticle surrounding pink laminated keratin, most consistent with a diagnosis of subungual onycholemmal cyst (SOC)(Figure 2). A reexcision was performed with removal of half of the nail bed, including a portion of the distal matrix extending inferiorly to the bone. Variably sized, epithelium-lined, keratin-filled cystic structures emanated from the nail bed epithelium. There were foci of hemorrhage and granulation tissue secondary to cyst rupture (Figure 3). The defect healed by secondary intention. No clinical evidence of recurrence was seen at 6-month follow-up.

Figure 2. Histopathology revealed a cystic structure with an epithelial lining with an intermittent focal granular layer and eosinophilic cuticle surrounding pink laminated keratin, most consistent with a diagnosis of a subungual onycholemmal cyst (A and B).

Figure 3. The gross specimen after reexcision revealed multiple foci of hemorrhage and brown keratinaceous material (A). Scanning magnification revealed variably sized, epithelium-lined, keratin-filled cystic structures emanating from the nail bed epithelium containing foci of calcification. There was hemorrhage and granulation tissue secondary to cyst rupture (B)(H&E, original magnification ×20).
Comment

Subungual onycholemmal cysts, also known as subungual epidermoid cysts or subungual epidermoid inclusions, are rare and distinctive nail abnormalities occurring in the dermis of the nail bed. We present a case of an SOC in a toenail mimicking subungual malignant melanoma.

Originally described by Samman1 in 1959, SOCs were attributed to trauma to the nail with resultant implantation of the epidermis into the deeper tissue. Lewin2,3 examined 90 postmortem fingernail and nail bed samples and found 8 subungual epidermoid cysts associated with clubbing of the fingernails. He postulated that the early pathogenesis of clubbing involved dermal fibroblast proliferation in the nail bed, leading to sequestration of nail bed epithelium into the dermis with resultant cyst formation. Microscopic subungual cysts also were identified in normal-appearing nails without evidence of trauma, thought to have arisen from the tips of the nail bed rete ridges by a process of bulbous proliferation rather than sequestration. These findings in normal nails suggest that SOCs may represent a more common entity than previously recognized.

 

 

It is imperative to recognize the presence of nail unit tumors early because of the risk for permanent nail plate dystrophy and the possibility of a malignant tumor.4,5 Subungual onycholemmal cysts may present with a wide spectrum of clinical findings including marked subungual hyperkeratosis, onychodystrophy, ridging, nail bed pigmentation, clubbing, thickening, or less often a normal-appearing nail. Based on reported cases, several trends are evident. Although nail dystrophy is most often asymptomatic, pain is not uncommon.5,6 It most commonly involves single digits, predominantly thumbs and great toenails.7,8 This predilection suggests that trauma or other local factors may be involved in its pathogenesis. Of note, trauma to the nail may occur years before the development of the lesions or it may not be recalled at all.

Diagnosis requires a degree of clinical suspicion and a nail bed biopsy with partial or total nail plate avulsion to visualize the pathologic portion of the nail bed. Because surgical intervention may lead to the implantation of epithelium, recurrences after nail biopsy or excision may occur.

In contrast to epidermal inclusion cysts arising in the skin, most SOCs do not have a granular layer.9 Hair and nails represent analogous differentiation products of the ectoderm. The nail matrix is homologous to portions of the hair matrix, while the nail bed epithelium is comparable to the outer root sheath of the hair follicle.7 Subungual onycholemmal cysts originate from the nail bed epithelium, which keratinizes in the absence of a granular layer, similar to the follicular isthmus outer root sheath. Thus, SOCs are comparable to the outer root sheath–derived isthmus-catagen cysts because of their abrupt central keratinization.8

Subungual onycholemmal cysts also must be distinguished from slowly growing malignant tumors of the nail bed epithelium, referred to as onycholemmal carcinomas by Alessi et al.10 This entity characteristically presents in elderly patients as a slowly growing, circumscribed, subungual discoloration that may ulcerate, destroying the nail apparatus and penetrating the phalangeal bone. On histopathology, it is characterized by small cysts filled with eosinophilic keratin devoid of a granular layer and lined by atypical squamous epithelium accompanied by solid nests and strands of atypical keratinocytes within the dermis.11 When a cystic component and clear cells predominate, the designation of malignant proliferating onycholemmal cyst has been applied. Its infiltrative growth pattern with destruction of the underlying bone makes it an important entity to exclude when considering the differential diagnosis of tumors of the nail bed.

Subungual melanomas comprise only 1% to 3% of malignant melanomas and 85% are initially misdiagnosed due to their rarity and nonspecific variable presentation. Aside from clinical evidence of Hutchinson sign in the early stages in almost all cases, accurate diagnosis of subungual melanoma and differentiation from SOCs relies on histopathology. A biopsy is necessary to make the diagnosis, but even microscopic findings may be nonspecific during the early stages.

Conclusion

We report a case of a 23-year-old woman with horizontal ridging and tenderness of the right great toenail associated with pigmentation of 5 years’ duration due to an SOC. The etiology of these subungual cysts, with or without nail abnormalities, still remains unclear. Its predilection for the thumbs and great toenails suggests that trauma or other local factors may be involved in its pathogenesis. Because of the rarity of this entity, there are no guidelines for surgical treatment. Subungual onycholemmal cysts may be an underrecognized and more common entity that must be considered when discussing tumors of the nail unit.

References
  1. Samman PD. The human toe nail. its genesis and blood supply. Br J Dermatol. 1959;71:296-302.
  2. Lewin K. The normal fingernail. Br J Dermatol. 1965;77:421-430.
  3. Lewin K. Subungual epidermoid inclusions. Br J Dermatol. 1969;81:671-675.
  4. Dominguez-Cherit J, Chanussot-Deprez C, Maria-Sarti H, et al. Nail unit tumors: a study of 234 patients in the dermatology department of the “Dr. Manuel Gea González” General Hospital in Mexico City. Dermatol Surg. 2008;34:1363-1371.
  5. Sáez-de-Ocariz MM, Domínguez-Cherit J, García-Corona C. Subungual epidermoid cysts. Int J Dermatol. 2001;40:524-526.
  6. Molly DO, Herbert K. Subungual epidermoid cyst. J Hand Surg Br. 2006;31:345.
  7. Telang GH, Jellinek N. Multiple calcified subungual epidermoid inclusions. J Am Acad Dermatol. 2007;56:336-339.
  8. Fanti PA, Tosti A. Subungual epidermoid inclusions: report of 8 cases. Dermatologica. 1989;178:209-212.
  9. Takiyoshi N, Nakano H, Matsuzaki T, et al. An eclipse in the subungual space: a diagnostic sign for a subungual epidermal cyst? Br J Dermatol. 2009;161:962-963.
  10. Alessi E, Coggi A, Gianotti R, et al. Onycholemmal carcinoma. Am J Dermatopathol. 2004;26:397-402.
  11. Inaoki M, Makino E, Adachi M, et al. Onycholemmal carcinoma. J Cutan Pathol. 2006;33:577-580.
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Drs. Busquets, Campanelli, Sahu, and Lee are from the Department of Dermatology and Cutaneous Biology, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania. Dr. Banala is from Jefferson Medical College, Thomas Jefferson University, Philadelphia.

The authors report no conflict of interest.

Correspondence: Joanna Busquets, MD, Thomas Jefferson University Hospital, Department of Dermatology and Cutaneous Biology, 833 Chestnut St, Ste 740, Philadelphia, PA 19107 ([email protected]).

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The authors report no conflict of interest.

Correspondence: Joanna Busquets, MD, Thomas Jefferson University Hospital, Department of Dermatology and Cutaneous Biology, 833 Chestnut St, Ste 740, Philadelphia, PA 19107 ([email protected]).

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Drs. Busquets, Campanelli, Sahu, and Lee are from the Department of Dermatology and Cutaneous Biology, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania. Dr. Banala is from Jefferson Medical College, Thomas Jefferson University, Philadelphia.

The authors report no conflict of interest.

Correspondence: Joanna Busquets, MD, Thomas Jefferson University Hospital, Department of Dermatology and Cutaneous Biology, 833 Chestnut St, Ste 740, Philadelphia, PA 19107 ([email protected]).

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Case Report

A 23-year-old woman presented with a horizontal split along the midline of the right great toenail associated with some tenderness of 2 to 3 months’ duration. Approximately 5 years prior, she noticed a bluish-colored area under the nail that had been steadily increasing in size. She denied a history of trauma, drainage, or bleeding. There was no history of other nail abnormalities. Her medications and personal, family, and social history were noncontributory.

Physical examination of the right great toenail revealed a horizontal split of the nail plate with a bluish hue visible under the nail plate (Figure 1A). The remaining toenails and fingernails were normal. A punch biopsy of the nail bed was performed with a presumptive clinical diagnosis of subungual melanoma versus melanocytic nevus versus cyst (Figure 1B). Nail plate avulsion revealed a blackened nail bed dotted with areas of bluish color and a red friable nodule present focally. Upon further inspection, extension was apparent into the distal matrix.

Figure 1. Subungual pressure-induced onycholysis overlying bluish discoloration of the lateral third of the right great toenail. The proximal and lateral nail folds were unaffected (A). A 4-mm punch biopsy site was visible on the nail bed following partial nail avulsion (B).

Histopathologic examination revealed a cystic structure with an epithelial lining mostly reminiscent of an isthmus catagen cyst admixed with the presence of both an intermittent focal granular layer and an eosinophilic cuticle surrounding pink laminated keratin, most consistent with a diagnosis of subungual onycholemmal cyst (SOC)(Figure 2). A reexcision was performed with removal of half of the nail bed, including a portion of the distal matrix extending inferiorly to the bone. Variably sized, epithelium-lined, keratin-filled cystic structures emanated from the nail bed epithelium. There were foci of hemorrhage and granulation tissue secondary to cyst rupture (Figure 3). The defect healed by secondary intention. No clinical evidence of recurrence was seen at 6-month follow-up.

Figure 2. Histopathology revealed a cystic structure with an epithelial lining with an intermittent focal granular layer and eosinophilic cuticle surrounding pink laminated keratin, most consistent with a diagnosis of a subungual onycholemmal cyst (A and B).

Figure 3. The gross specimen after reexcision revealed multiple foci of hemorrhage and brown keratinaceous material (A). Scanning magnification revealed variably sized, epithelium-lined, keratin-filled cystic structures emanating from the nail bed epithelium containing foci of calcification. There was hemorrhage and granulation tissue secondary to cyst rupture (B)(H&E, original magnification ×20).
Comment

Subungual onycholemmal cysts, also known as subungual epidermoid cysts or subungual epidermoid inclusions, are rare and distinctive nail abnormalities occurring in the dermis of the nail bed. We present a case of an SOC in a toenail mimicking subungual malignant melanoma.

Originally described by Samman1 in 1959, SOCs were attributed to trauma to the nail with resultant implantation of the epidermis into the deeper tissue. Lewin2,3 examined 90 postmortem fingernail and nail bed samples and found 8 subungual epidermoid cysts associated with clubbing of the fingernails. He postulated that the early pathogenesis of clubbing involved dermal fibroblast proliferation in the nail bed, leading to sequestration of nail bed epithelium into the dermis with resultant cyst formation. Microscopic subungual cysts also were identified in normal-appearing nails without evidence of trauma, thought to have arisen from the tips of the nail bed rete ridges by a process of bulbous proliferation rather than sequestration. These findings in normal nails suggest that SOCs may represent a more common entity than previously recognized.

 

 

It is imperative to recognize the presence of nail unit tumors early because of the risk for permanent nail plate dystrophy and the possibility of a malignant tumor.4,5 Subungual onycholemmal cysts may present with a wide spectrum of clinical findings including marked subungual hyperkeratosis, onychodystrophy, ridging, nail bed pigmentation, clubbing, thickening, or less often a normal-appearing nail. Based on reported cases, several trends are evident. Although nail dystrophy is most often asymptomatic, pain is not uncommon.5,6 It most commonly involves single digits, predominantly thumbs and great toenails.7,8 This predilection suggests that trauma or other local factors may be involved in its pathogenesis. Of note, trauma to the nail may occur years before the development of the lesions or it may not be recalled at all.

Diagnosis requires a degree of clinical suspicion and a nail bed biopsy with partial or total nail plate avulsion to visualize the pathologic portion of the nail bed. Because surgical intervention may lead to the implantation of epithelium, recurrences after nail biopsy or excision may occur.

In contrast to epidermal inclusion cysts arising in the skin, most SOCs do not have a granular layer.9 Hair and nails represent analogous differentiation products of the ectoderm. The nail matrix is homologous to portions of the hair matrix, while the nail bed epithelium is comparable to the outer root sheath of the hair follicle.7 Subungual onycholemmal cysts originate from the nail bed epithelium, which keratinizes in the absence of a granular layer, similar to the follicular isthmus outer root sheath. Thus, SOCs are comparable to the outer root sheath–derived isthmus-catagen cysts because of their abrupt central keratinization.8

Subungual onycholemmal cysts also must be distinguished from slowly growing malignant tumors of the nail bed epithelium, referred to as onycholemmal carcinomas by Alessi et al.10 This entity characteristically presents in elderly patients as a slowly growing, circumscribed, subungual discoloration that may ulcerate, destroying the nail apparatus and penetrating the phalangeal bone. On histopathology, it is characterized by small cysts filled with eosinophilic keratin devoid of a granular layer and lined by atypical squamous epithelium accompanied by solid nests and strands of atypical keratinocytes within the dermis.11 When a cystic component and clear cells predominate, the designation of malignant proliferating onycholemmal cyst has been applied. Its infiltrative growth pattern with destruction of the underlying bone makes it an important entity to exclude when considering the differential diagnosis of tumors of the nail bed.

Subungual melanomas comprise only 1% to 3% of malignant melanomas and 85% are initially misdiagnosed due to their rarity and nonspecific variable presentation. Aside from clinical evidence of Hutchinson sign in the early stages in almost all cases, accurate diagnosis of subungual melanoma and differentiation from SOCs relies on histopathology. A biopsy is necessary to make the diagnosis, but even microscopic findings may be nonspecific during the early stages.

Conclusion

We report a case of a 23-year-old woman with horizontal ridging and tenderness of the right great toenail associated with pigmentation of 5 years’ duration due to an SOC. The etiology of these subungual cysts, with or without nail abnormalities, still remains unclear. Its predilection for the thumbs and great toenails suggests that trauma or other local factors may be involved in its pathogenesis. Because of the rarity of this entity, there are no guidelines for surgical treatment. Subungual onycholemmal cysts may be an underrecognized and more common entity that must be considered when discussing tumors of the nail unit.

Case Report

A 23-year-old woman presented with a horizontal split along the midline of the right great toenail associated with some tenderness of 2 to 3 months’ duration. Approximately 5 years prior, she noticed a bluish-colored area under the nail that had been steadily increasing in size. She denied a history of trauma, drainage, or bleeding. There was no history of other nail abnormalities. Her medications and personal, family, and social history were noncontributory.

Physical examination of the right great toenail revealed a horizontal split of the nail plate with a bluish hue visible under the nail plate (Figure 1A). The remaining toenails and fingernails were normal. A punch biopsy of the nail bed was performed with a presumptive clinical diagnosis of subungual melanoma versus melanocytic nevus versus cyst (Figure 1B). Nail plate avulsion revealed a blackened nail bed dotted with areas of bluish color and a red friable nodule present focally. Upon further inspection, extension was apparent into the distal matrix.

Figure 1. Subungual pressure-induced onycholysis overlying bluish discoloration of the lateral third of the right great toenail. The proximal and lateral nail folds were unaffected (A). A 4-mm punch biopsy site was visible on the nail bed following partial nail avulsion (B).

Histopathologic examination revealed a cystic structure with an epithelial lining mostly reminiscent of an isthmus catagen cyst admixed with the presence of both an intermittent focal granular layer and an eosinophilic cuticle surrounding pink laminated keratin, most consistent with a diagnosis of subungual onycholemmal cyst (SOC)(Figure 2). A reexcision was performed with removal of half of the nail bed, including a portion of the distal matrix extending inferiorly to the bone. Variably sized, epithelium-lined, keratin-filled cystic structures emanated from the nail bed epithelium. There were foci of hemorrhage and granulation tissue secondary to cyst rupture (Figure 3). The defect healed by secondary intention. No clinical evidence of recurrence was seen at 6-month follow-up.

Figure 2. Histopathology revealed a cystic structure with an epithelial lining with an intermittent focal granular layer and eosinophilic cuticle surrounding pink laminated keratin, most consistent with a diagnosis of a subungual onycholemmal cyst (A and B).

Figure 3. The gross specimen after reexcision revealed multiple foci of hemorrhage and brown keratinaceous material (A). Scanning magnification revealed variably sized, epithelium-lined, keratin-filled cystic structures emanating from the nail bed epithelium containing foci of calcification. There was hemorrhage and granulation tissue secondary to cyst rupture (B)(H&E, original magnification ×20).
Comment

Subungual onycholemmal cysts, also known as subungual epidermoid cysts or subungual epidermoid inclusions, are rare and distinctive nail abnormalities occurring in the dermis of the nail bed. We present a case of an SOC in a toenail mimicking subungual malignant melanoma.

Originally described by Samman1 in 1959, SOCs were attributed to trauma to the nail with resultant implantation of the epidermis into the deeper tissue. Lewin2,3 examined 90 postmortem fingernail and nail bed samples and found 8 subungual epidermoid cysts associated with clubbing of the fingernails. He postulated that the early pathogenesis of clubbing involved dermal fibroblast proliferation in the nail bed, leading to sequestration of nail bed epithelium into the dermis with resultant cyst formation. Microscopic subungual cysts also were identified in normal-appearing nails without evidence of trauma, thought to have arisen from the tips of the nail bed rete ridges by a process of bulbous proliferation rather than sequestration. These findings in normal nails suggest that SOCs may represent a more common entity than previously recognized.

 

 

It is imperative to recognize the presence of nail unit tumors early because of the risk for permanent nail plate dystrophy and the possibility of a malignant tumor.4,5 Subungual onycholemmal cysts may present with a wide spectrum of clinical findings including marked subungual hyperkeratosis, onychodystrophy, ridging, nail bed pigmentation, clubbing, thickening, or less often a normal-appearing nail. Based on reported cases, several trends are evident. Although nail dystrophy is most often asymptomatic, pain is not uncommon.5,6 It most commonly involves single digits, predominantly thumbs and great toenails.7,8 This predilection suggests that trauma or other local factors may be involved in its pathogenesis. Of note, trauma to the nail may occur years before the development of the lesions or it may not be recalled at all.

Diagnosis requires a degree of clinical suspicion and a nail bed biopsy with partial or total nail plate avulsion to visualize the pathologic portion of the nail bed. Because surgical intervention may lead to the implantation of epithelium, recurrences after nail biopsy or excision may occur.

In contrast to epidermal inclusion cysts arising in the skin, most SOCs do not have a granular layer.9 Hair and nails represent analogous differentiation products of the ectoderm. The nail matrix is homologous to portions of the hair matrix, while the nail bed epithelium is comparable to the outer root sheath of the hair follicle.7 Subungual onycholemmal cysts originate from the nail bed epithelium, which keratinizes in the absence of a granular layer, similar to the follicular isthmus outer root sheath. Thus, SOCs are comparable to the outer root sheath–derived isthmus-catagen cysts because of their abrupt central keratinization.8

Subungual onycholemmal cysts also must be distinguished from slowly growing malignant tumors of the nail bed epithelium, referred to as onycholemmal carcinomas by Alessi et al.10 This entity characteristically presents in elderly patients as a slowly growing, circumscribed, subungual discoloration that may ulcerate, destroying the nail apparatus and penetrating the phalangeal bone. On histopathology, it is characterized by small cysts filled with eosinophilic keratin devoid of a granular layer and lined by atypical squamous epithelium accompanied by solid nests and strands of atypical keratinocytes within the dermis.11 When a cystic component and clear cells predominate, the designation of malignant proliferating onycholemmal cyst has been applied. Its infiltrative growth pattern with destruction of the underlying bone makes it an important entity to exclude when considering the differential diagnosis of tumors of the nail bed.

Subungual melanomas comprise only 1% to 3% of malignant melanomas and 85% are initially misdiagnosed due to their rarity and nonspecific variable presentation. Aside from clinical evidence of Hutchinson sign in the early stages in almost all cases, accurate diagnosis of subungual melanoma and differentiation from SOCs relies on histopathology. A biopsy is necessary to make the diagnosis, but even microscopic findings may be nonspecific during the early stages.

Conclusion

We report a case of a 23-year-old woman with horizontal ridging and tenderness of the right great toenail associated with pigmentation of 5 years’ duration due to an SOC. The etiology of these subungual cysts, with or without nail abnormalities, still remains unclear. Its predilection for the thumbs and great toenails suggests that trauma or other local factors may be involved in its pathogenesis. Because of the rarity of this entity, there are no guidelines for surgical treatment. Subungual onycholemmal cysts may be an underrecognized and more common entity that must be considered when discussing tumors of the nail unit.

References
  1. Samman PD. The human toe nail. its genesis and blood supply. Br J Dermatol. 1959;71:296-302.
  2. Lewin K. The normal fingernail. Br J Dermatol. 1965;77:421-430.
  3. Lewin K. Subungual epidermoid inclusions. Br J Dermatol. 1969;81:671-675.
  4. Dominguez-Cherit J, Chanussot-Deprez C, Maria-Sarti H, et al. Nail unit tumors: a study of 234 patients in the dermatology department of the “Dr. Manuel Gea González” General Hospital in Mexico City. Dermatol Surg. 2008;34:1363-1371.
  5. Sáez-de-Ocariz MM, Domínguez-Cherit J, García-Corona C. Subungual epidermoid cysts. Int J Dermatol. 2001;40:524-526.
  6. Molly DO, Herbert K. Subungual epidermoid cyst. J Hand Surg Br. 2006;31:345.
  7. Telang GH, Jellinek N. Multiple calcified subungual epidermoid inclusions. J Am Acad Dermatol. 2007;56:336-339.
  8. Fanti PA, Tosti A. Subungual epidermoid inclusions: report of 8 cases. Dermatologica. 1989;178:209-212.
  9. Takiyoshi N, Nakano H, Matsuzaki T, et al. An eclipse in the subungual space: a diagnostic sign for a subungual epidermal cyst? Br J Dermatol. 2009;161:962-963.
  10. Alessi E, Coggi A, Gianotti R, et al. Onycholemmal carcinoma. Am J Dermatopathol. 2004;26:397-402.
  11. Inaoki M, Makino E, Adachi M, et al. Onycholemmal carcinoma. J Cutan Pathol. 2006;33:577-580.
References
  1. Samman PD. The human toe nail. its genesis and blood supply. Br J Dermatol. 1959;71:296-302.
  2. Lewin K. The normal fingernail. Br J Dermatol. 1965;77:421-430.
  3. Lewin K. Subungual epidermoid inclusions. Br J Dermatol. 1969;81:671-675.
  4. Dominguez-Cherit J, Chanussot-Deprez C, Maria-Sarti H, et al. Nail unit tumors: a study of 234 patients in the dermatology department of the “Dr. Manuel Gea González” General Hospital in Mexico City. Dermatol Surg. 2008;34:1363-1371.
  5. Sáez-de-Ocariz MM, Domínguez-Cherit J, García-Corona C. Subungual epidermoid cysts. Int J Dermatol. 2001;40:524-526.
  6. Molly DO, Herbert K. Subungual epidermoid cyst. J Hand Surg Br. 2006;31:345.
  7. Telang GH, Jellinek N. Multiple calcified subungual epidermoid inclusions. J Am Acad Dermatol. 2007;56:336-339.
  8. Fanti PA, Tosti A. Subungual epidermoid inclusions: report of 8 cases. Dermatologica. 1989;178:209-212.
  9. Takiyoshi N, Nakano H, Matsuzaki T, et al. An eclipse in the subungual space: a diagnostic sign for a subungual epidermal cyst? Br J Dermatol. 2009;161:962-963.
  10. Alessi E, Coggi A, Gianotti R, et al. Onycholemmal carcinoma. Am J Dermatopathol. 2004;26:397-402.
  11. Inaoki M, Makino E, Adachi M, et al. Onycholemmal carcinoma. J Cutan Pathol. 2006;33:577-580.
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Practice Points

  • Trauma to the nail may occur years before the development of subungual onycholemmal cysts or it may not be recalled at all.
  • Diagnosis requires a degree of clinical suspicion and a nail bed biopsy.
  • Subungual onycholemmal cysts must be distinguished from slowly growing malignant tumors of the nail bed epithelium.
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Tofacitinib clears pediatric alopecia areata in small study

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Tofacitinib clears pediatric alopecia areata in small study

MINNEAPOLIS – The first study to evaluate tofacitinib’s effectiveness at treating severe alopecia areata in the pediatric population found that the janus kinase inhibitor was effective for more than half of the patients, and well tolerated by all.

Of a case series of 13 pediatric patients who had alopecia areata (AA) and were treated with tofacitinib, 9 (68%) experienced “clinically significant” regrowth of hair, with mean improvement in the Severity of Alopecia Tool (SALT) score of 88% for these responders. The nonresponding group, all of whom had alopecia universalis or totalis, saw essentially no response, with a 1% reduction in SALT score.

Lucy Y. Liu, a medical student at Yale University, New Haven, Conn., presented the findings at the annual meeting of the Society for Pediatric Dermatology.

Ms. Liu and her coinvestigators reported that all of the patients had severe AA by SALT scoring, with an overall mean pretreatment SALT score of 74. Eight of the patients (62%) had alopecia universalis, and two (15%) had alopecia totalis.

The patients ranged in age from 12 to 17 years, with a median age of 15. All but three were male, and patients were an average 9 years old at onset of AA. For patients with alopecia totalis or universalis, the duration of the current episode was a median 1.75 years.

Five patients (38%) had atopic dermatitis, while 1 (8%) had thyroid disease. Three patients (23%) had family members with AA; all but one patient, however, had a family history of autoimmune disease of some sort.

Patients were given tofacitinib 5 mg orally twice daily for 5 months. One patient developed new patches of alopecia during treatment, so the dosing for that patient was increased to 10 mg/5 mg daily.

Adverse events for participants included headaches, upper respiratory infections, and “mild, transient increases in transaminases,” wrote Dr. Lieu and her collaborators. No serious adverse events were reported.

Previous work at Yale had shown that tofacitinib reversed alopecia universalis in a patient who received the medication for plaque psoriasis, and that topical treatment with ruxolitinib, another janus kinase inhibitor, was effective in treating alopecia universalis.

Study limitations included the small sample size and the relatively short duration of follow-up, an important consideration because relapse has been observed after tofacitinib treatment in AA. Still, “Tofacitinib is a promising therapy for the treatment of severe AA in adolescents,” wrote Ms. Liu and her colleagues, recommending randomized clinical trials for further exploration of efficacy and safety in the pediatric population.

[email protected]

On Twitter @karioakes

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MINNEAPOLIS – The first study to evaluate tofacitinib’s effectiveness at treating severe alopecia areata in the pediatric population found that the janus kinase inhibitor was effective for more than half of the patients, and well tolerated by all.

Of a case series of 13 pediatric patients who had alopecia areata (AA) and were treated with tofacitinib, 9 (68%) experienced “clinically significant” regrowth of hair, with mean improvement in the Severity of Alopecia Tool (SALT) score of 88% for these responders. The nonresponding group, all of whom had alopecia universalis or totalis, saw essentially no response, with a 1% reduction in SALT score.

Lucy Y. Liu, a medical student at Yale University, New Haven, Conn., presented the findings at the annual meeting of the Society for Pediatric Dermatology.

Ms. Liu and her coinvestigators reported that all of the patients had severe AA by SALT scoring, with an overall mean pretreatment SALT score of 74. Eight of the patients (62%) had alopecia universalis, and two (15%) had alopecia totalis.

The patients ranged in age from 12 to 17 years, with a median age of 15. All but three were male, and patients were an average 9 years old at onset of AA. For patients with alopecia totalis or universalis, the duration of the current episode was a median 1.75 years.

Five patients (38%) had atopic dermatitis, while 1 (8%) had thyroid disease. Three patients (23%) had family members with AA; all but one patient, however, had a family history of autoimmune disease of some sort.

Patients were given tofacitinib 5 mg orally twice daily for 5 months. One patient developed new patches of alopecia during treatment, so the dosing for that patient was increased to 10 mg/5 mg daily.

Adverse events for participants included headaches, upper respiratory infections, and “mild, transient increases in transaminases,” wrote Dr. Lieu and her collaborators. No serious adverse events were reported.

Previous work at Yale had shown that tofacitinib reversed alopecia universalis in a patient who received the medication for plaque psoriasis, and that topical treatment with ruxolitinib, another janus kinase inhibitor, was effective in treating alopecia universalis.

Study limitations included the small sample size and the relatively short duration of follow-up, an important consideration because relapse has been observed after tofacitinib treatment in AA. Still, “Tofacitinib is a promising therapy for the treatment of severe AA in adolescents,” wrote Ms. Liu and her colleagues, recommending randomized clinical trials for further exploration of efficacy and safety in the pediatric population.

[email protected]

On Twitter @karioakes

MINNEAPOLIS – The first study to evaluate tofacitinib’s effectiveness at treating severe alopecia areata in the pediatric population found that the janus kinase inhibitor was effective for more than half of the patients, and well tolerated by all.

Of a case series of 13 pediatric patients who had alopecia areata (AA) and were treated with tofacitinib, 9 (68%) experienced “clinically significant” regrowth of hair, with mean improvement in the Severity of Alopecia Tool (SALT) score of 88% for these responders. The nonresponding group, all of whom had alopecia universalis or totalis, saw essentially no response, with a 1% reduction in SALT score.

Lucy Y. Liu, a medical student at Yale University, New Haven, Conn., presented the findings at the annual meeting of the Society for Pediatric Dermatology.

Ms. Liu and her coinvestigators reported that all of the patients had severe AA by SALT scoring, with an overall mean pretreatment SALT score of 74. Eight of the patients (62%) had alopecia universalis, and two (15%) had alopecia totalis.

The patients ranged in age from 12 to 17 years, with a median age of 15. All but three were male, and patients were an average 9 years old at onset of AA. For patients with alopecia totalis or universalis, the duration of the current episode was a median 1.75 years.

Five patients (38%) had atopic dermatitis, while 1 (8%) had thyroid disease. Three patients (23%) had family members with AA; all but one patient, however, had a family history of autoimmune disease of some sort.

Patients were given tofacitinib 5 mg orally twice daily for 5 months. One patient developed new patches of alopecia during treatment, so the dosing for that patient was increased to 10 mg/5 mg daily.

Adverse events for participants included headaches, upper respiratory infections, and “mild, transient increases in transaminases,” wrote Dr. Lieu and her collaborators. No serious adverse events were reported.

Previous work at Yale had shown that tofacitinib reversed alopecia universalis in a patient who received the medication for plaque psoriasis, and that topical treatment with ruxolitinib, another janus kinase inhibitor, was effective in treating alopecia universalis.

Study limitations included the small sample size and the relatively short duration of follow-up, an important consideration because relapse has been observed after tofacitinib treatment in AA. Still, “Tofacitinib is a promising therapy for the treatment of severe AA in adolescents,” wrote Ms. Liu and her colleagues, recommending randomized clinical trials for further exploration of efficacy and safety in the pediatric population.

[email protected]

On Twitter @karioakes

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Key clinical point: The JAK inhibitor tofacitinib resolved alopecia areata in 68% of pediatric patients.

Major finding: Among the responders, the Severity of Alopecia Tool score improved by a mean 88% over 5 months.

Data source: Case series of 13 pediatric patients with severe alopecia areata treated with tofacitinib at a single site.

Disclosures: Ms. Liu and her collaborators reported no conflicts of interest.