Cutis

To the Editor:

Herpes vegetans (HV) is an uncommon infection caused by human herpesvirus (HHV) in patients who are immunocompromised, such as those who are HIV positive.¹ Unlike typical HHV infection, HV can present with exophytic exudative ulcers and papillomatous vegetations. The presentation of ulcerated genital nodules, especially in an immunocompromised patient, yields an array of disorders in the differential diagnosis, including condyloma latum, condyloma acuminatum, pyogenic granuloma (PG), and verrucous carcinoma.^2,3 Histopathology of HV reveals pseudoepitheliomatous hyperplasia, plasma cell infiltration, and positivity for HHV type 1 (HHV-1) and/or HHV type 2 (HHV-2). Herpes vegetans lesions typically require a multimodal treatment approach because many cases are resistant to acyclovir. Treatment options include the nucleoside analogues foscarnet and cidofovir; immunomodulators such as topical imiquimod; and the topical antiviral trifluridine.^1,4-6 We describe a case of HV in a patient with a history of well-controlled HIV infection who presented with a painful fungating penile lesion.

A 55-year-old man presented to the hospital with a painful expanding mass on the distal aspect of the penis of 3 months’ duration. He had a history of HIV infection that was well-controlled by antiretroviral therapy, prior hepatitis B virus infection and acyclovir-resistant genital HHV-2 infection. Physical examination revealed a large, firm, circumferential, exophytic, verrucous plaque with various areas of ulceration and purulent drainage on the distal shaft and glans of the penis (Figure 1). The patient’s most recent absolute CD4 count was 425 cells/mm³ (reference range, 500–1500 cells/mm³). His HIV viral load was undetectable at less than 30 copies/mL. Histopathology with hematoxylin and eosin staining of biopsy material from the penile lesion demonstrated pseudoepitheliomatous epidermal hyperplasia with focal ulceration and a mixed inflammatory infiltrate (Figure 2A). At higher magnification, clear viral cytopathic changes of HHV were noted, including multinucleation, nuclear molding, and homogenous gray nuclei (Figure 2B). Additional staining for fungi, mycobacteria, and spirochetes was negative. In-situ hybridization was negative for human papillomavirus subtypes. A bacterial culture of swabs of the purulent drainage was positive for Staphylococcus aureus and Proteus mirabilis.

Given the patient’s known history of acyclovir-resistant HHV-2 infection, he received a 28-day course of intravenous foscarnet 40 mg/kg every 12 hours. He also was given a 14-day course of intravenous ampicillin-sulbactam 3 g every 6 hours. The patient gradually improved during a 35-day hospital stay. He was discharged with cidofovir cream 1% and oral valacyclovir; the latter was subsequently discontinued by dermatology because of his known history of acyclovir resistance. Four months after discharge, the patient underwent a circumcision performed by urology to decrease the risk for recurrence and achieve the best cosmetic outcome. At the 6-month follow-up visit, dramatic clinical improvement was evident, with complete resolution of the plaque and only isolated areas of scarring (Figure 3). The patient reported that penile function was preserved.

Herpes vegetans represents a rare infection with HHV-1 or HHV-2, typically in patients who are considerably immunosuppressed, such as those with cancer, those undergoing transplantation, and those with uncontrolled HIV infection.¹ Few cases of HV have been described in an immunocompetent patient.² Our case is unique because the patient’s HIV infection was well controlled at the time HV was diagnosed, demonstrated by his modestly low CD4 count and undetectable HIV viral load.

Patients with HV can present diagnostic and therapeutic challenges. Typically, a diagnosis of cutaneous HHV infection does not require a biopsy; most cases appear as clustered vesicular lesions, making the disease easy to diagnose clinically. However, biopsies and cultures are necessary to identify the underlying cause of atypical verrucous exophytic lesions. Other conditions with clinical features similar to HV include squamous cell carcinoma, condyloma acuminatum, and deep fungal and mycobacterial infections.^2,3 A tissue biopsy, histologic staining, and tissue culture should be performed to identify the causative pathogen and potential targets for treatment. Definitive diagnosis is vital to deliver proper treatment modalities, which often involve a multimodal multidisciplinary approach. 

Several pathogenic mechanisms of HV have been proposed. One theory suggests that in an immunocompetent patient, HHV typically triggers a lymphocytic response, which leads to activation of interferon alpha. However, in an immunocompromised patient, such as an individual with AIDS, this interferon response is diminished, which explains why these patients typically have a chronic and resistant HHV infection. HIV has an affinity for infecting dermal dendritic cells, which signals activation of tumor necrosis factor and interleukin.⁶ Both cytokines contribute to an antiapoptotic environment that promotes continued proliferation of these viral cells in the epidermis. Over time, propagation of disinhibited cells can lead to the verrucous and hyperkeratotic-appearing skin that is common in patients with HV.⁷

Another theorized mechanism underlying hypertrophic herpetic lesions was described in the context of HHV-1 infection and subsequent PG. El Hayderi et al⁸ reported that histologic and immunohistochemical examination of a patient’s lesion revealed sparse epithelial cell aggregates within PG as well as HHV-1 antigens in the nuclei and cytoplasm of normal-appearing and cytopathic epithelial cells. Immunohistochemical examination also revealed vascular endothelial growth factor within HHV-1–infected epithelial cells and PG endothelial cells, suggesting that PG formation may be indirectly driven by vascular endothelial growth factor and its proangiogenic properties. The pathogenesis of PG in the setting of HHV-1 infection displays many similarities to hyperkeratotic lesions observed in atypical cutaneous manifestations of HHV-2.⁸

The management of patients with HV continues to be complex, often requiring a multimodal regimen. Although acyclovir has been shown to be highly effective for treating and preventing most HHV infections, acyclovir resistance frequently has been reported in immunocompromised populations.⁵ Acyclovir resistance can be correlated with the severity of immunodeficiency as well as the duration of acyclovir exposure. Resistance to acyclovir often results from deficient intracellular phosphorylation, which is required for activation of the drug. If patients show resistance to acyclovir and its derivatives, alternate drug classes that do not depend on thymidine kinase phosphorylation should be considered.

Our patient received a combination of intravenous foscarnet and a course of ampicillin-sulbactam while an inpatient due to his documented history of acyclovir-resistant HHV-2 infection, and he was discharged on cidofovir cream 1%. Cidofovir is US Food and Drug Administration approved for treating cytomegalovirus retinitis in patients with AIDS. Although data are limited, topical and intralesional cidofovir have been used to treat acyclovir-resistant cases of HV with documented success.^1,9 In refractory HV or when the disease is slow to resolve, intralesional cidofovir has been documented to be an additional treatment option. Intralesional and topical cidofovir carry a much lower risk for adverse effects such as kidney dysfunction compared to intravenous cidofovir¹ and can be considered in patients with minimal clinical improvement and those at increased risk for side effects.

Our case demonstrated how a patient with HV may require a complex and prolonged hospital course for appropriate treatment. Our patient required an array of both medical and surgical modalities to reach the desired outcome. Here, a multitude of specialties including infectious disease, dermatology, and urology worked together to reach a positive clinical and cosmetic outcome for this patient. 

To the Editor:

Herpes vegetans (HV) is an uncommon infection caused by human herpesvirus (HHV) in patients who are immunocompromised, such as those who are HIV positive.¹ Unlike typical HHV infection, HV can present with exophytic exudative ulcers and papillomatous vegetations. The presentation of ulcerated genital nodules, especially in an immunocompromised patient, yields an array of disorders in the differential diagnosis, including condyloma latum, condyloma acuminatum, pyogenic granuloma (PG), and verrucous carcinoma.^2,3 Histopathology of HV reveals pseudoepitheliomatous hyperplasia, plasma cell infiltration, and positivity for HHV type 1 (HHV-1) and/or HHV type 2 (HHV-2). Herpes vegetans lesions typically require a multimodal treatment approach because many cases are resistant to acyclovir. Treatment options include the nucleoside analogues foscarnet and cidofovir; immunomodulators such as topical imiquimod; and the topical antiviral trifluridine.^1,4-6 We describe a case of HV in a patient with a history of well-controlled HIV infection who presented with a painful fungating penile lesion.

A 55-year-old man presented to the hospital with a painful expanding mass on the distal aspect of the penis of 3 months’ duration. He had a history of HIV infection that was well-controlled by antiretroviral therapy, prior hepatitis B virus infection and acyclovir-resistant genital HHV-2 infection. Physical examination revealed a large, firm, circumferential, exophytic, verrucous plaque with various areas of ulceration and purulent drainage on the distal shaft and glans of the penis (Figure 1). The patient’s most recent absolute CD4 count was 425 cells/mm³ (reference range, 500–1500 cells/mm³). His HIV viral load was undetectable at less than 30 copies/mL. Histopathology with hematoxylin and eosin staining of biopsy material from the penile lesion demonstrated pseudoepitheliomatous epidermal hyperplasia with focal ulceration and a mixed inflammatory infiltrate (Figure 2A). At higher magnification, clear viral cytopathic changes of HHV were noted, including multinucleation, nuclear molding, and homogenous gray nuclei (Figure 2B). Additional staining for fungi, mycobacteria, and spirochetes was negative. In-situ hybridization was negative for human papillomavirus subtypes. A bacterial culture of swabs of the purulent drainage was positive for Staphylococcus aureus and Proteus mirabilis.

Given the patient’s known history of acyclovir-resistant HHV-2 infection, he received a 28-day course of intravenous foscarnet 40 mg/kg every 12 hours. He also was given a 14-day course of intravenous ampicillin-sulbactam 3 g every 6 hours. The patient gradually improved during a 35-day hospital stay. He was discharged with cidofovir cream 1% and oral valacyclovir; the latter was subsequently discontinued by dermatology because of his known history of acyclovir resistance. Four months after discharge, the patient underwent a circumcision performed by urology to decrease the risk for recurrence and achieve the best cosmetic outcome. At the 6-month follow-up visit, dramatic clinical improvement was evident, with complete resolution of the plaque and only isolated areas of scarring (Figure 3). The patient reported that penile function was preserved.

Herpes vegetans represents a rare infection with HHV-1 or HHV-2, typically in patients who are considerably immunosuppressed, such as those with cancer, those undergoing transplantation, and those with uncontrolled HIV infection.¹ Few cases of HV have been described in an immunocompetent patient.² Our case is unique because the patient’s HIV infection was well controlled at the time HV was diagnosed, demonstrated by his modestly low CD4 count and undetectable HIV viral load.

Patients with HV can present diagnostic and therapeutic challenges. Typically, a diagnosis of cutaneous HHV infection does not require a biopsy; most cases appear as clustered vesicular lesions, making the disease easy to diagnose clinically. However, biopsies and cultures are necessary to identify the underlying cause of atypical verrucous exophytic lesions. Other conditions with clinical features similar to HV include squamous cell carcinoma, condyloma acuminatum, and deep fungal and mycobacterial infections.^2,3 A tissue biopsy, histologic staining, and tissue culture should be performed to identify the causative pathogen and potential targets for treatment. Definitive diagnosis is vital to deliver proper treatment modalities, which often involve a multimodal multidisciplinary approach. 

Several pathogenic mechanisms of HV have been proposed. One theory suggests that in an immunocompetent patient, HHV typically triggers a lymphocytic response, which leads to activation of interferon alpha. However, in an immunocompromised patient, such as an individual with AIDS, this interferon response is diminished, which explains why these patients typically have a chronic and resistant HHV infection. HIV has an affinity for infecting dermal dendritic cells, which signals activation of tumor necrosis factor and interleukin.⁶ Both cytokines contribute to an antiapoptotic environment that promotes continued proliferation of these viral cells in the epidermis. Over time, propagation of disinhibited cells can lead to the verrucous and hyperkeratotic-appearing skin that is common in patients with HV.⁷

Another theorized mechanism underlying hypertrophic herpetic lesions was described in the context of HHV-1 infection and subsequent PG. El Hayderi et al⁸ reported that histologic and immunohistochemical examination of a patient’s lesion revealed sparse epithelial cell aggregates within PG as well as HHV-1 antigens in the nuclei and cytoplasm of normal-appearing and cytopathic epithelial cells. Immunohistochemical examination also revealed vascular endothelial growth factor within HHV-1–infected epithelial cells and PG endothelial cells, suggesting that PG formation may be indirectly driven by vascular endothelial growth factor and its proangiogenic properties. The pathogenesis of PG in the setting of HHV-1 infection displays many similarities to hyperkeratotic lesions observed in atypical cutaneous manifestations of HHV-2.⁸

The management of patients with HV continues to be complex, often requiring a multimodal regimen. Although acyclovir has been shown to be highly effective for treating and preventing most HHV infections, acyclovir resistance frequently has been reported in immunocompromised populations.⁵ Acyclovir resistance can be correlated with the severity of immunodeficiency as well as the duration of acyclovir exposure. Resistance to acyclovir often results from deficient intracellular phosphorylation, which is required for activation of the drug. If patients show resistance to acyclovir and its derivatives, alternate drug classes that do not depend on thymidine kinase phosphorylation should be considered.

Our patient received a combination of intravenous foscarnet and a course of ampicillin-sulbactam while an inpatient due to his documented history of acyclovir-resistant HHV-2 infection, and he was discharged on cidofovir cream 1%. Cidofovir is US Food and Drug Administration approved for treating cytomegalovirus retinitis in patients with AIDS. Although data are limited, topical and intralesional cidofovir have been used to treat acyclovir-resistant cases of HV with documented success.^1,9 In refractory HV or when the disease is slow to resolve, intralesional cidofovir has been documented to be an additional treatment option. Intralesional and topical cidofovir carry a much lower risk for adverse effects such as kidney dysfunction compared to intravenous cidofovir¹ and can be considered in patients with minimal clinical improvement and those at increased risk for side effects.

Our case demonstrated how a patient with HV may require a complex and prolonged hospital course for appropriate treatment. Our patient required an array of both medical and surgical modalities to reach the desired outcome. Here, a multitude of specialties including infectious disease, dermatology, and urology worked together to reach a positive clinical and cosmetic outcome for this patient. 

To the Editor:

Herpes vegetans (HV) is an uncommon infection caused by human herpesvirus (HHV) in patients who are immunocompromised, such as those who are HIV positive.¹ Unlike typical HHV infection, HV can present with exophytic exudative ulcers and papillomatous vegetations. The presentation of ulcerated genital nodules, especially in an immunocompromised patient, yields an array of disorders in the differential diagnosis, including condyloma latum, condyloma acuminatum, pyogenic granuloma (PG), and verrucous carcinoma.^2,3 Histopathology of HV reveals pseudoepitheliomatous hyperplasia, plasma cell infiltration, and positivity for HHV type 1 (HHV-1) and/or HHV type 2 (HHV-2). Herpes vegetans lesions typically require a multimodal treatment approach because many cases are resistant to acyclovir. Treatment options include the nucleoside analogues foscarnet and cidofovir; immunomodulators such as topical imiquimod; and the topical antiviral trifluridine.^1,4-6 We describe a case of HV in a patient with a history of well-controlled HIV infection who presented with a painful fungating penile lesion.

A 55-year-old man presented to the hospital with a painful expanding mass on the distal aspect of the penis of 3 months’ duration. He had a history of HIV infection that was well-controlled by antiretroviral therapy, prior hepatitis B virus infection and acyclovir-resistant genital HHV-2 infection. Physical examination revealed a large, firm, circumferential, exophytic, verrucous plaque with various areas of ulceration and purulent drainage on the distal shaft and glans of the penis (Figure 1). The patient’s most recent absolute CD4 count was 425 cells/mm³ (reference range, 500–1500 cells/mm³). His HIV viral load was undetectable at less than 30 copies/mL. Histopathology with hematoxylin and eosin staining of biopsy material from the penile lesion demonstrated pseudoepitheliomatous epidermal hyperplasia with focal ulceration and a mixed inflammatory infiltrate (Figure 2A). At higher magnification, clear viral cytopathic changes of HHV were noted, including multinucleation, nuclear molding, and homogenous gray nuclei (Figure 2B). Additional staining for fungi, mycobacteria, and spirochetes was negative. In-situ hybridization was negative for human papillomavirus subtypes. A bacterial culture of swabs of the purulent drainage was positive for Staphylococcus aureus and Proteus mirabilis.

Given the patient’s known history of acyclovir-resistant HHV-2 infection, he received a 28-day course of intravenous foscarnet 40 mg/kg every 12 hours. He also was given a 14-day course of intravenous ampicillin-sulbactam 3 g every 6 hours. The patient gradually improved during a 35-day hospital stay. He was discharged with cidofovir cream 1% and oral valacyclovir; the latter was subsequently discontinued by dermatology because of his known history of acyclovir resistance. Four months after discharge, the patient underwent a circumcision performed by urology to decrease the risk for recurrence and achieve the best cosmetic outcome. At the 6-month follow-up visit, dramatic clinical improvement was evident, with complete resolution of the plaque and only isolated areas of scarring (Figure 3). The patient reported that penile function was preserved.

Herpes vegetans represents a rare infection with HHV-1 or HHV-2, typically in patients who are considerably immunosuppressed, such as those with cancer, those undergoing transplantation, and those with uncontrolled HIV infection.¹ Few cases of HV have been described in an immunocompetent patient.² Our case is unique because the patient’s HIV infection was well controlled at the time HV was diagnosed, demonstrated by his modestly low CD4 count and undetectable HIV viral load.

Patients with HV can present diagnostic and therapeutic challenges. Typically, a diagnosis of cutaneous HHV infection does not require a biopsy; most cases appear as clustered vesicular lesions, making the disease easy to diagnose clinically. However, biopsies and cultures are necessary to identify the underlying cause of atypical verrucous exophytic lesions. Other conditions with clinical features similar to HV include squamous cell carcinoma, condyloma acuminatum, and deep fungal and mycobacterial infections.^2,3 A tissue biopsy, histologic staining, and tissue culture should be performed to identify the causative pathogen and potential targets for treatment. Definitive diagnosis is vital to deliver proper treatment modalities, which often involve a multimodal multidisciplinary approach. 

Several pathogenic mechanisms of HV have been proposed. One theory suggests that in an immunocompetent patient, HHV typically triggers a lymphocytic response, which leads to activation of interferon alpha. However, in an immunocompromised patient, such as an individual with AIDS, this interferon response is diminished, which explains why these patients typically have a chronic and resistant HHV infection. HIV has an affinity for infecting dermal dendritic cells, which signals activation of tumor necrosis factor and interleukin.⁶ Both cytokines contribute to an antiapoptotic environment that promotes continued proliferation of these viral cells in the epidermis. Over time, propagation of disinhibited cells can lead to the verrucous and hyperkeratotic-appearing skin that is common in patients with HV.⁷

Another theorized mechanism underlying hypertrophic herpetic lesions was described in the context of HHV-1 infection and subsequent PG. El Hayderi et al⁸ reported that histologic and immunohistochemical examination of a patient’s lesion revealed sparse epithelial cell aggregates within PG as well as HHV-1 antigens in the nuclei and cytoplasm of normal-appearing and cytopathic epithelial cells. Immunohistochemical examination also revealed vascular endothelial growth factor within HHV-1–infected epithelial cells and PG endothelial cells, suggesting that PG formation may be indirectly driven by vascular endothelial growth factor and its proangiogenic properties. The pathogenesis of PG in the setting of HHV-1 infection displays many similarities to hyperkeratotic lesions observed in atypical cutaneous manifestations of HHV-2.⁸

The management of patients with HV continues to be complex, often requiring a multimodal regimen. Although acyclovir has been shown to be highly effective for treating and preventing most HHV infections, acyclovir resistance frequently has been reported in immunocompromised populations.⁵ Acyclovir resistance can be correlated with the severity of immunodeficiency as well as the duration of acyclovir exposure. Resistance to acyclovir often results from deficient intracellular phosphorylation, which is required for activation of the drug. If patients show resistance to acyclovir and its derivatives, alternate drug classes that do not depend on thymidine kinase phosphorylation should be considered.

Our patient received a combination of intravenous foscarnet and a course of ampicillin-sulbactam while an inpatient due to his documented history of acyclovir-resistant HHV-2 infection, and he was discharged on cidofovir cream 1%. Cidofovir is US Food and Drug Administration approved for treating cytomegalovirus retinitis in patients with AIDS. Although data are limited, topical and intralesional cidofovir have been used to treat acyclovir-resistant cases of HV with documented success.^1,9 In refractory HV or when the disease is slow to resolve, intralesional cidofovir has been documented to be an additional treatment option. Intralesional and topical cidofovir carry a much lower risk for adverse effects such as kidney dysfunction compared to intravenous cidofovir¹ and can be considered in patients with minimal clinical improvement and those at increased risk for side effects.

Our case demonstrated how a patient with HV may require a complex and prolonged hospital course for appropriate treatment. Our patient required an array of both medical and surgical modalities to reach the desired outcome. Here, a multitude of specialties including infectious disease, dermatology, and urology worked together to reach a positive clinical and cosmetic outcome for this patient. 

To the Editor:

Most cutaneous squamous cell carcinomas (cSCCs) are successfully treated with standard modalities such as surgical excision; however, a subset of tumors is not amenable to surgical resection.^1,2 Patients who are not able to undergo surgical treatment may instead receive radiation therapy, topical 5-fluorouracil (5-FU), imiquimod, cryosurgery, photodynamic therapy, or systemic treatment (eg, immunotherapy) in addition to intralesional approaches for localized disease.^1-4 However, the adverse effects associated with these treatments and their modest effect in preventing the recurrence of cutaneous lesions limit their efficacy against unresectable cSCC.^4-6 We present a case that demonstrates the efficacy of electrodesiccation and curettage (ED&C) followed by topical 5-FU for an invasive cSCC not amenable to surgical therapy.

A 58-year-old woman presented for evaluation of a 3.5×3.4-cm, incisional biopsy–proven, invasive stage T2a cSCC (Brigham and Women’s Hospital tumor staging system [Boston, Massachusetts]) on the dorsal aspect of the left foot, which had developed over several months (Figure 1A). She had a history of treatment with psoralen plus UV light therapy for erythroderma of unknown cause and peripheral neuropathy. She was not a surgical candidate because of suspected underlying cutaneous sclerosis and a history of poor wound healing on the lower legs.

Prior to presentation to dermatology, the patient had been treated with intralesional methotrexate, intralesional 5-FU, and the antiangiogenic and antiproliferative combination agent OLCAT-005³—consisting of equal parts [by volume] of diclofenac gel 3%, imiquimod cream 5%, hydrocortisone valerate cream 0.2%, calcipotriene cream 0.005%, and tretinoin cream 0.05—which failed, and the patient reported that OLCAT-005 made the pain from the cSCC worse.

Upon growth of the lesion over several months, the patient was referred to the High-Risk Skin Cancer Clinic at Massachusetts General Hospital (Boston, Massachusetts). A repeat biopsy demonstrated an invasive well-differentiated cSCC (Figure 2). The size and invasive features of the lesion on clinical examination prompted a referral to surgical oncology for a wide local excision. However, surgical oncology concluded she was not a surgical candidate.

Magnetic resonance imaging showed no deep invasion of the cSCC to the tendons or bones. Electrodesiccation and curettage was performed to debulk the tumor, followed by twice-daily application of topical 5-FU for 4 weeks to improve the odds of tumor clearance (Figure 1B). Fourteen weeks after completion of 5-FU treatment, the cSCC showed complete clinical regression (Figure 1C). No recurrence has been detected clinically more than 3 years following treatment.

Prior to the advent of Mohs micrographic surgery, ED&C commonly was used to treat skin cancer, with a lower cost and a cure rate close to 95%.^7,8 We postulate that the mechanism of tumor regression in our patient was ED&C-mediated removal and necrosis of neoplastic tissue combined with 5-FU–induced cancer-cell DNA damage and apoptosis. An antitumor immune response also may have contributed to the complete regression of the cSCC.

Although antiangiogenic and antiproliferative agents are suitable for primary cSCC treatment, it is possible that this patient’s prior therapies alone—in the absence of debulking by ED&C to sufficiently reduce disease burden—did not allow for tumor clearance and were ineffective. Many clinicians are reluctant to apply 5-FU to a wound bed because it can impede wound healing.⁹ In this case, re-epithelialization likely occurred primarily after completion of 5-FU treatment.

We recommend consideration of ED&C with 5-FU for similar malignant lesions that are not amenable to surgical excision. Nevertheless, Mohs micrographic surgery and wide local excision remain the gold standards for definitive treatment of invasive skin cancer in a patient who is a candidate for surgical treatment.

To the Editor:

Most cutaneous squamous cell carcinomas (cSCCs) are successfully treated with standard modalities such as surgical excision; however, a subset of tumors is not amenable to surgical resection.^1,2 Patients who are not able to undergo surgical treatment may instead receive radiation therapy, topical 5-fluorouracil (5-FU), imiquimod, cryosurgery, photodynamic therapy, or systemic treatment (eg, immunotherapy) in addition to intralesional approaches for localized disease.^1-4 However, the adverse effects associated with these treatments and their modest effect in preventing the recurrence of cutaneous lesions limit their efficacy against unresectable cSCC.^4-6 We present a case that demonstrates the efficacy of electrodesiccation and curettage (ED&C) followed by topical 5-FU for an invasive cSCC not amenable to surgical therapy.

A 58-year-old woman presented for evaluation of a 3.5×3.4-cm, incisional biopsy–proven, invasive stage T2a cSCC (Brigham and Women’s Hospital tumor staging system [Boston, Massachusetts]) on the dorsal aspect of the left foot, which had developed over several months (Figure 1A). She had a history of treatment with psoralen plus UV light therapy for erythroderma of unknown cause and peripheral neuropathy. She was not a surgical candidate because of suspected underlying cutaneous sclerosis and a history of poor wound healing on the lower legs.

Prior to presentation to dermatology, the patient had been treated with intralesional methotrexate, intralesional 5-FU, and the antiangiogenic and antiproliferative combination agent OLCAT-005³—consisting of equal parts [by volume] of diclofenac gel 3%, imiquimod cream 5%, hydrocortisone valerate cream 0.2%, calcipotriene cream 0.005%, and tretinoin cream 0.05—which failed, and the patient reported that OLCAT-005 made the pain from the cSCC worse.

Upon growth of the lesion over several months, the patient was referred to the High-Risk Skin Cancer Clinic at Massachusetts General Hospital (Boston, Massachusetts). A repeat biopsy demonstrated an invasive well-differentiated cSCC (Figure 2). The size and invasive features of the lesion on clinical examination prompted a referral to surgical oncology for a wide local excision. However, surgical oncology concluded she was not a surgical candidate.

Magnetic resonance imaging showed no deep invasion of the cSCC to the tendons or bones. Electrodesiccation and curettage was performed to debulk the tumor, followed by twice-daily application of topical 5-FU for 4 weeks to improve the odds of tumor clearance (Figure 1B). Fourteen weeks after completion of 5-FU treatment, the cSCC showed complete clinical regression (Figure 1C). No recurrence has been detected clinically more than 3 years following treatment.

Prior to the advent of Mohs micrographic surgery, ED&C commonly was used to treat skin cancer, with a lower cost and a cure rate close to 95%.^7,8 We postulate that the mechanism of tumor regression in our patient was ED&C-mediated removal and necrosis of neoplastic tissue combined with 5-FU–induced cancer-cell DNA damage and apoptosis. An antitumor immune response also may have contributed to the complete regression of the cSCC.

Although antiangiogenic and antiproliferative agents are suitable for primary cSCC treatment, it is possible that this patient’s prior therapies alone—in the absence of debulking by ED&C to sufficiently reduce disease burden—did not allow for tumor clearance and were ineffective. Many clinicians are reluctant to apply 5-FU to a wound bed because it can impede wound healing.⁹ In this case, re-epithelialization likely occurred primarily after completion of 5-FU treatment.

We recommend consideration of ED&C with 5-FU for similar malignant lesions that are not amenable to surgical excision. Nevertheless, Mohs micrographic surgery and wide local excision remain the gold standards for definitive treatment of invasive skin cancer in a patient who is a candidate for surgical treatment.

To the Editor:

Most cutaneous squamous cell carcinomas (cSCCs) are successfully treated with standard modalities such as surgical excision; however, a subset of tumors is not amenable to surgical resection.^1,2 Patients who are not able to undergo surgical treatment may instead receive radiation therapy, topical 5-fluorouracil (5-FU), imiquimod, cryosurgery, photodynamic therapy, or systemic treatment (eg, immunotherapy) in addition to intralesional approaches for localized disease.^1-4 However, the adverse effects associated with these treatments and their modest effect in preventing the recurrence of cutaneous lesions limit their efficacy against unresectable cSCC.^4-6 We present a case that demonstrates the efficacy of electrodesiccation and curettage (ED&C) followed by topical 5-FU for an invasive cSCC not amenable to surgical therapy.

A 58-year-old woman presented for evaluation of a 3.5×3.4-cm, incisional biopsy–proven, invasive stage T2a cSCC (Brigham and Women’s Hospital tumor staging system [Boston, Massachusetts]) on the dorsal aspect of the left foot, which had developed over several months (Figure 1A). She had a history of treatment with psoralen plus UV light therapy for erythroderma of unknown cause and peripheral neuropathy. She was not a surgical candidate because of suspected underlying cutaneous sclerosis and a history of poor wound healing on the lower legs.

Prior to presentation to dermatology, the patient had been treated with intralesional methotrexate, intralesional 5-FU, and the antiangiogenic and antiproliferative combination agent OLCAT-005³—consisting of equal parts [by volume] of diclofenac gel 3%, imiquimod cream 5%, hydrocortisone valerate cream 0.2%, calcipotriene cream 0.005%, and tretinoin cream 0.05—which failed, and the patient reported that OLCAT-005 made the pain from the cSCC worse.

Upon growth of the lesion over several months, the patient was referred to the High-Risk Skin Cancer Clinic at Massachusetts General Hospital (Boston, Massachusetts). A repeat biopsy demonstrated an invasive well-differentiated cSCC (Figure 2). The size and invasive features of the lesion on clinical examination prompted a referral to surgical oncology for a wide local excision. However, surgical oncology concluded she was not a surgical candidate.

Magnetic resonance imaging showed no deep invasion of the cSCC to the tendons or bones. Electrodesiccation and curettage was performed to debulk the tumor, followed by twice-daily application of topical 5-FU for 4 weeks to improve the odds of tumor clearance (Figure 1B). Fourteen weeks after completion of 5-FU treatment, the cSCC showed complete clinical regression (Figure 1C). No recurrence has been detected clinically more than 3 years following treatment.

Prior to the advent of Mohs micrographic surgery, ED&C commonly was used to treat skin cancer, with a lower cost and a cure rate close to 95%.^7,8 We postulate that the mechanism of tumor regression in our patient was ED&C-mediated removal and necrosis of neoplastic tissue combined with 5-FU–induced cancer-cell DNA damage and apoptosis. An antitumor immune response also may have contributed to the complete regression of the cSCC.

Although antiangiogenic and antiproliferative agents are suitable for primary cSCC treatment, it is possible that this patient’s prior therapies alone—in the absence of debulking by ED&C to sufficiently reduce disease burden—did not allow for tumor clearance and were ineffective. Many clinicians are reluctant to apply 5-FU to a wound bed because it can impede wound healing.⁹ In this case, re-epithelialization likely occurred primarily after completion of 5-FU treatment.

We recommend consideration of ED&C with 5-FU for similar malignant lesions that are not amenable to surgical excision. Nevertheless, Mohs micrographic surgery and wide local excision remain the gold standards for definitive treatment of invasive skin cancer in a patient who is a candidate for surgical treatment.

The Diagnosis: Extragenital Lichen Sclerosus et Atrophicus

A punch biopsy of the lesion revealed epidermal hyperkeratosis, atrophy, follicular plugs with basal vacuolar degeneration, and homogenous dense fibrosis in the papillary dermis with a dense lymphocytic infiltrate beneath the fibrosis (Figure 1). Dermoscopic examination was remarkable for a distinctive rainbow pattern. Clinical, histopathologic, and dermoscopic findings led to the diagnosis of extragenital lichen sclerosus et atrophicus (LSEA). A potent corticosteroid cream was prescribed twice daily for 2 months, after which the lesions completely resolved. At 2-year follow-up, a relapse was not observed (Figure 2).

Lichen sclerosus et atrophicus is an inflammatory dermatosis that clinically presents as atrophic or hypertrophic plaques that may show pigmentation changes with anogenital and extragenital involvement. It is common among females and predominantly occurs in prepubescent girls and postmenopausal women. The exact etiology is unclear; however, it is hypothesized to occur secondary to autoimmunity with an underlying genetic predisposition. Local trauma, hormonal influences, and infections are other suspected etiologic factors. Genital lesions often lead to itching, pain, and dyspareunia, whereas extragenital lesions predominantly are asymptomatic. When symptomatic, itching usually is the main concern. Unlike genital LSEA, extragenital lesions are not associated with squamous cell carcinoma development. Reported dermoscopic features of LSEA are white structureless areas with scaling, comedolike openings, follicular plugs, white shiny streaks, blue-gray peppering, pigment network, and red-purple globules.¹ In our case, the dermoscopic finding of a rainbow pattern in LSEA is rare.² Although the mechanism behind this appearance unclear, it can be the result of the birefringence effect—local variations in refractive index—influenced by the direction of structures within the dermis such as collagen. In this case, there was diffuse and dense homogenous fibrosis in the superficial dermis that corresponded to dermoscopic white polygonal clods.

Extragenital LSEA commonly is located on the neck, shoulders, wrists, and upper trunk and manifests clinically as whitish papules coalescing into scarlike plaques. Of all patients who have LSEA, 20% have extragenital lesions, and most of these lesions are seen in patients who also have genital LSEA. Approximately 6% of all LSEA patients have extragenital LSEA without genital involvement.³

For experienced dermatologists, clinical symptoms and lesion characteristics usually are sufficient for diagnosis; however, a differential diagnosis of atypical lesions and isolated extragenital presentations such as morphea, lichen simplex chronicus, lichen planus, and vitiligo requires the correlation of clinical findings with histopathology and dermoscopy. Morphea, known as localized scleroderma, is an idiopathic inflammatory skin disease with sclerotic changes. It manifests as inflammatory plaques that vary in color from red to purple. If there is moderate sclerosis in the center of this plaque, the color progressively fades to white, leaving a purplish ring around the edges. Dermoscopic features of morphea are reported as areas of erythema; red-focused vessels of linear, irregular, or dotted morphology; white fibrotic beams; and pigmentary structures.² Lichen simplex chronicus is characterized by single or multiple dry and patchy skin lesions that are intensely pruritic. It commonly occurs on the neck, scalp, extremities, genital areas, and buttocks. Scratching the lesions leads to scarring, thickening of the skin, and increased frequency of itching. Histopathology of lichen simplex chronicus most frequently demonstrates a thickening of the epidermis and papillary dermis, irregularly elongated rete ridges, and fibroplasia with stellate or multinucleated fibroblasts completed by perivascular lymphocytic inflammation.⁴ Lichen planus presents with pruritic, polygonal, purple papules and/or plaques that can present in a variety of clinical forms, including atrophic and hypertrophic lichen planus.⁵ Lichen planus was an unlikely diagnosis for our patient due to the presence of patchy scarlike lesions and dermoscopic features that are well described in patients with LSEA. Lichen sclerosus et atrophicus presents with hypopigmented and/or hyperpigmented patches and plaques, distinguishing itself from vitiligo, which has flat lesions.

Topical steroids are the first-line therapeutic agents in the treatment of LSEA.6 Despite frequent use in this setting, common side effects such as localized scarring and atrophic degenerations have led to debate about their use. In our patient, the lesions resolved almost completely in 2 months, and no relapse was observed in the following 2 years. In the setting of topical steroid resistance, topical calcineurin inhibitors, UVA/UVB phototherapy, and topical tacrolimus can be used for treatment.⁶

The diagnosis of isolated extragenital LSEA may be a clinical challenge and generally requires further workup. When evaluating extragenital lesions, dermatologists should keep in mind extragenital LSEA as a differential diagnosis in the presence of a dermoscopic rainbow pattern arranged over white polygonal clods.

The Diagnosis: Extragenital Lichen Sclerosus et Atrophicus

A punch biopsy of the lesion revealed epidermal hyperkeratosis, atrophy, follicular plugs with basal vacuolar degeneration, and homogenous dense fibrosis in the papillary dermis with a dense lymphocytic infiltrate beneath the fibrosis (Figure 1). Dermoscopic examination was remarkable for a distinctive rainbow pattern. Clinical, histopathologic, and dermoscopic findings led to the diagnosis of extragenital lichen sclerosus et atrophicus (LSEA). A potent corticosteroid cream was prescribed twice daily for 2 months, after which the lesions completely resolved. At 2-year follow-up, a relapse was not observed (Figure 2).

Lichen sclerosus et atrophicus is an inflammatory dermatosis that clinically presents as atrophic or hypertrophic plaques that may show pigmentation changes with anogenital and extragenital involvement. It is common among females and predominantly occurs in prepubescent girls and postmenopausal women. The exact etiology is unclear; however, it is hypothesized to occur secondary to autoimmunity with an underlying genetic predisposition. Local trauma, hormonal influences, and infections are other suspected etiologic factors. Genital lesions often lead to itching, pain, and dyspareunia, whereas extragenital lesions predominantly are asymptomatic. When symptomatic, itching usually is the main concern. Unlike genital LSEA, extragenital lesions are not associated with squamous cell carcinoma development. Reported dermoscopic features of LSEA are white structureless areas with scaling, comedolike openings, follicular plugs, white shiny streaks, blue-gray peppering, pigment network, and red-purple globules.¹ In our case, the dermoscopic finding of a rainbow pattern in LSEA is rare.² Although the mechanism behind this appearance unclear, it can be the result of the birefringence effect—local variations in refractive index—influenced by the direction of structures within the dermis such as collagen. In this case, there was diffuse and dense homogenous fibrosis in the superficial dermis that corresponded to dermoscopic white polygonal clods.

Extragenital LSEA commonly is located on the neck, shoulders, wrists, and upper trunk and manifests clinically as whitish papules coalescing into scarlike plaques. Of all patients who have LSEA, 20% have extragenital lesions, and most of these lesions are seen in patients who also have genital LSEA. Approximately 6% of all LSEA patients have extragenital LSEA without genital involvement.³

For experienced dermatologists, clinical symptoms and lesion characteristics usually are sufficient for diagnosis; however, a differential diagnosis of atypical lesions and isolated extragenital presentations such as morphea, lichen simplex chronicus, lichen planus, and vitiligo requires the correlation of clinical findings with histopathology and dermoscopy. Morphea, known as localized scleroderma, is an idiopathic inflammatory skin disease with sclerotic changes. It manifests as inflammatory plaques that vary in color from red to purple. If there is moderate sclerosis in the center of this plaque, the color progressively fades to white, leaving a purplish ring around the edges. Dermoscopic features of morphea are reported as areas of erythema; red-focused vessels of linear, irregular, or dotted morphology; white fibrotic beams; and pigmentary structures.² Lichen simplex chronicus is characterized by single or multiple dry and patchy skin lesions that are intensely pruritic. It commonly occurs on the neck, scalp, extremities, genital areas, and buttocks. Scratching the lesions leads to scarring, thickening of the skin, and increased frequency of itching. Histopathology of lichen simplex chronicus most frequently demonstrates a thickening of the epidermis and papillary dermis, irregularly elongated rete ridges, and fibroplasia with stellate or multinucleated fibroblasts completed by perivascular lymphocytic inflammation.⁴ Lichen planus presents with pruritic, polygonal, purple papules and/or plaques that can present in a variety of clinical forms, including atrophic and hypertrophic lichen planus.⁵ Lichen planus was an unlikely diagnosis for our patient due to the presence of patchy scarlike lesions and dermoscopic features that are well described in patients with LSEA. Lichen sclerosus et atrophicus presents with hypopigmented and/or hyperpigmented patches and plaques, distinguishing itself from vitiligo, which has flat lesions.

Topical steroids are the first-line therapeutic agents in the treatment of LSEA.6 Despite frequent use in this setting, common side effects such as localized scarring and atrophic degenerations have led to debate about their use. In our patient, the lesions resolved almost completely in 2 months, and no relapse was observed in the following 2 years. In the setting of topical steroid resistance, topical calcineurin inhibitors, UVA/UVB phototherapy, and topical tacrolimus can be used for treatment.⁶

The diagnosis of isolated extragenital LSEA may be a clinical challenge and generally requires further workup. When evaluating extragenital lesions, dermatologists should keep in mind extragenital LSEA as a differential diagnosis in the presence of a dermoscopic rainbow pattern arranged over white polygonal clods.

The Diagnosis: Extragenital Lichen Sclerosus et Atrophicus

A punch biopsy of the lesion revealed epidermal hyperkeratosis, atrophy, follicular plugs with basal vacuolar degeneration, and homogenous dense fibrosis in the papillary dermis with a dense lymphocytic infiltrate beneath the fibrosis (Figure 1). Dermoscopic examination was remarkable for a distinctive rainbow pattern. Clinical, histopathologic, and dermoscopic findings led to the diagnosis of extragenital lichen sclerosus et atrophicus (LSEA). A potent corticosteroid cream was prescribed twice daily for 2 months, after which the lesions completely resolved. At 2-year follow-up, a relapse was not observed (Figure 2).

Lichen sclerosus et atrophicus is an inflammatory dermatosis that clinically presents as atrophic or hypertrophic plaques that may show pigmentation changes with anogenital and extragenital involvement. It is common among females and predominantly occurs in prepubescent girls and postmenopausal women. The exact etiology is unclear; however, it is hypothesized to occur secondary to autoimmunity with an underlying genetic predisposition. Local trauma, hormonal influences, and infections are other suspected etiologic factors. Genital lesions often lead to itching, pain, and dyspareunia, whereas extragenital lesions predominantly are asymptomatic. When symptomatic, itching usually is the main concern. Unlike genital LSEA, extragenital lesions are not associated with squamous cell carcinoma development. Reported dermoscopic features of LSEA are white structureless areas with scaling, comedolike openings, follicular plugs, white shiny streaks, blue-gray peppering, pigment network, and red-purple globules.¹ In our case, the dermoscopic finding of a rainbow pattern in LSEA is rare.² Although the mechanism behind this appearance unclear, it can be the result of the birefringence effect—local variations in refractive index—influenced by the direction of structures within the dermis such as collagen. In this case, there was diffuse and dense homogenous fibrosis in the superficial dermis that corresponded to dermoscopic white polygonal clods.

Extragenital LSEA commonly is located on the neck, shoulders, wrists, and upper trunk and manifests clinically as whitish papules coalescing into scarlike plaques. Of all patients who have LSEA, 20% have extragenital lesions, and most of these lesions are seen in patients who also have genital LSEA. Approximately 6% of all LSEA patients have extragenital LSEA without genital involvement.³

For experienced dermatologists, clinical symptoms and lesion characteristics usually are sufficient for diagnosis; however, a differential diagnosis of atypical lesions and isolated extragenital presentations such as morphea, lichen simplex chronicus, lichen planus, and vitiligo requires the correlation of clinical findings with histopathology and dermoscopy. Morphea, known as localized scleroderma, is an idiopathic inflammatory skin disease with sclerotic changes. It manifests as inflammatory plaques that vary in color from red to purple. If there is moderate sclerosis in the center of this plaque, the color progressively fades to white, leaving a purplish ring around the edges. Dermoscopic features of morphea are reported as areas of erythema; red-focused vessels of linear, irregular, or dotted morphology; white fibrotic beams; and pigmentary structures.² Lichen simplex chronicus is characterized by single or multiple dry and patchy skin lesions that are intensely pruritic. It commonly occurs on the neck, scalp, extremities, genital areas, and buttocks. Scratching the lesions leads to scarring, thickening of the skin, and increased frequency of itching. Histopathology of lichen simplex chronicus most frequently demonstrates a thickening of the epidermis and papillary dermis, irregularly elongated rete ridges, and fibroplasia with stellate or multinucleated fibroblasts completed by perivascular lymphocytic inflammation.⁴ Lichen planus presents with pruritic, polygonal, purple papules and/or plaques that can present in a variety of clinical forms, including atrophic and hypertrophic lichen planus.⁵ Lichen planus was an unlikely diagnosis for our patient due to the presence of patchy scarlike lesions and dermoscopic features that are well described in patients with LSEA. Lichen sclerosus et atrophicus presents with hypopigmented and/or hyperpigmented patches and plaques, distinguishing itself from vitiligo, which has flat lesions.

Topical steroids are the first-line therapeutic agents in the treatment of LSEA.6 Despite frequent use in this setting, common side effects such as localized scarring and atrophic degenerations have led to debate about their use. In our patient, the lesions resolved almost completely in 2 months, and no relapse was observed in the following 2 years. In the setting of topical steroid resistance, topical calcineurin inhibitors, UVA/UVB phototherapy, and topical tacrolimus can be used for treatment.⁶

The diagnosis of isolated extragenital LSEA may be a clinical challenge and generally requires further workup. When evaluating extragenital lesions, dermatologists should keep in mind extragenital LSEA as a differential diagnosis in the presence of a dermoscopic rainbow pattern arranged over white polygonal clods.

To the Editor:

Porocarcinoma, or malignant poroma, is a rare adnexal malignancy of a predominantly glandular origin that comprises less than 0.01% of all cutaneous neoplasms.^1,2 Although exposure to UV radiation and immunosuppression have been implicated in the malignant degeneration of benign poromas into porocarcinomas, at least half of all malignant variants will arise de novo.^3,4 Patients present with an evolving nodule or plaque and often are in their seventh or eighth decade of life at the time of diagnosis.² Localized trauma from burns or radiation exposure has been causatively linked to de novo porocarcinoma formation.^2,5 These suppressive and traumatic stimuli drive increased genetic heterogeneity along with characteristic gene mutations in known tumor suppressor genes.⁶

A 62-year-old man presented with a nonhealing wound on the right hand of 5 years’ duration that had previously been attributed to a penetrating injury with a piece of copper from a refrigerant coolant system. The wound initially blistered and then eventually callused and developed areas of ulceration. The patient consulted multiple physicians for treatment of the intensely pruritic and ulcerated lesion. He received prescriptions for cephalexin, trimethoprim-sulfamethoxazole, doxycycline, clindamycin, and clobetasol cream, all of which offered minimal improvement. Home therapies including vitamin E and tea tree oil yielded no benefit. The lesion roughly quadrupled in size over the last 5 years.

Physical examination revealed a 7.5×4.2-cm ulcerated plaque with ragged borders and abundant central neoepithelialization on the right palmar surface (Figure 1). No gross motor or sensory defects were identified. There was no epitrochlear, axillary, cervical, or supraclavicular lymphadenopathy. A shave biopsy of the plaque’s edge was performed, which demonstrated a hyperplastic epidermis comprising atypical poroid cells with frequent mitoses, scant necrosis, and regular ductal structures confined to the epidermis (Figure 2). Immunohistochemical profiling results were positive for anticytokeratin (CAM 5.2) and Ber-EP4 (Figure 3). When evaluated in aggregate, these findings were consistent with porocarcinoma in situ.

The patient was referred to a surgical oncologist for evaluation. At that time, an exophytic mass had developed in the central lesion. Although no lymphadenopathy was identified upon examination, the patient had developed tremoring and a contracture deformity of the right hand. Extensive imaging and urgent surgical resection were recommended, but the patient did not wish to pursue these options, opting instead to continue home remedies. At a 15-month follow-up via telephone, the patient reported that the home therapy had failed and he had moved back to Vietnam. Partial limb amputation had been recommended by a local provider. Unfortunately, the patient was subsequently lost to follow-up, and his current status is unknown.

Porocarcinomas are rare tumors, comprising just 0.005% to 0.01% of all cutaneous epithelial tumors.^1,2,5 They affect men and women equally, with an average age at diagnosis of 60 to 70 years.^1,2 At least half of all porocarcinomas develop de novo, while 18% to 50% arise from the degeneration of an existing poroma.^2,3 Exposure to UV light and immunosuppression, particularly following organ transplantation, represent 2 commonly suspected catalysts for this malignant transformation.⁴ De novo porocarcinomas are most causatively linked to localized trauma from burns or radiation exposure.⁵ Gene mutations in classic tumor suppressor genes—tumor protein p53 (TP53), phosphatase and tensin homolog (PTEN), rearranged during transfection (RET), adenomatous polyposis coli (APC)—and increased genetic heterogeneity follow these stimuli.⁶

The morphologic presentation of porocarcinoma is highly variable and may manifest as papules, nodules, or plaques in various states of erosion, ulceration, or excoriation. Diagnoses of basal and squamous cell carcinoma, primary adnexal tumors, seborrheic keratosis, pyogenic granuloma, and melanoma must all be considered and methodically ruled out.⁷ Porocarcinomas may arise nearly anywhere on the body, with a particular predilection for the lower extremities (35%), head/neck (24%), and upper extremities (14%).^3,4 Primary lesions arising from the extremities, genitalia, or buttocks herald a higher risk for lymphatic invasion and distant metastasis, while head and neck tumors more commonly remain localized.⁸ Bleeding, ulceration, or rapid expansion of a preexisting poroma is suggestive of malignant transformation and may portend a more aggressive disease pattern.^2,9

Unequivocal diagnosis relies on histological and immunohistochemical studies due to the marked clinical variance of this neoplasm.⁷ An irregular histologic pattern of poromatous basaloid cells with ductal differentiation and cytologic atypia commonly are seen with porocarcinomas.^2,8 Nuclear pleomorphism with cellular necrosis, increased mitotic figures, and abortive ductal formation with a distinct lack of retraction around cellular aggregates often are found. Immunohistochemical staining is needed to confirm the primary tumor diagnosis. Histochemical stains commonly employed include carcinoembryonic antigen (CEA), cytokeratin AE1/AE3, epithelial membrane antigen, p53, p63, Ki67, and periodic acid-Schiff.¹⁰ The use of BerEP4 has been reported as efficacious in highlighting sweat structures, which can be particularly useful in cases when basal cell carcinoma is not in the histologic differential.¹¹ These staining profiles afford confirmation of ductal differentiation with CEA, epithelial membrane antigen, and BerEP4, while p63 and Ki67 are used as surrogates for primary cutaneous neoplasia and cell proliferation, respectively.^5,11 Porocarcinoma lesions may be most sensitive to CEA and most specific to CK19 (a component of cytokeratin AE1/AE3), though these findings have not been widely reproduced.⁷

The treatment and prognosis of porocarcinoma vary widely. Surgically excised lesions recur in roughly 20% of cases, though these rates likely include tumors that were incompletely resected in the primary attempt. Although wide local excision with an average 1-cm margin remains the most employed removal technique, Mohs micrographic surgery may more effectively limit recurrence and metastasis of localized disease.^7,8,12 Metastatic disease foretells a mortality rate of at least 65%, which is problematic in that 10% to 20% of patients have metastatic disease at the time of diagnosis and another 20% will show metastasis following primary tumor excision.^8,10 Neoplasms with high mitotic rates and depths greater than 7 mm should prompt thorough diagnostic imaging, such as positron emission tomography or magnetic resonance imaging. A sentinel lymph node biopsy should be strongly considered and discussed with the patient.¹⁰ Treatment options for nodal and distant metastases include a combination of localized surgery, lymphadenectomy, radiotherapy, and chemotherapeutic agents.^2,4,5 The response to systemic treatment and radiotherapy often is quite poor, though the use of combinations of docetaxel, paclitaxel, cetuximab, and immunotherapy have been efficacious in smaller studies.^8,10 The highest rates of morbidity and mortality are seen in patients with metastases on presentation or with localized tumors in the groin and buttocks.⁸

The diagnosis of porocarcinoma may be elusive due to its relatively rare occurrence. Therefore, it is critical to consider this neoplasm in high-risk sites in older patients who present with an evolving nodule or tumor on an extremity. Routine histology and astute histochemical profiling are necessary to exclude diseases that mimic porocarcinoma. Once diagnosis is confirmed, management with prompt excision and diagnostic imaging is recommended, including a lymph node biopsy if appropriate. Due to its high metastatic potential and associated morbidity and mortality, patients with porocarcinoma should be followed closely by a multidisciplinary care team.

To the Editor:

Porocarcinoma, or malignant poroma, is a rare adnexal malignancy of a predominantly glandular origin that comprises less than 0.01% of all cutaneous neoplasms.^1,2 Although exposure to UV radiation and immunosuppression have been implicated in the malignant degeneration of benign poromas into porocarcinomas, at least half of all malignant variants will arise de novo.^3,4 Patients present with an evolving nodule or plaque and often are in their seventh or eighth decade of life at the time of diagnosis.² Localized trauma from burns or radiation exposure has been causatively linked to de novo porocarcinoma formation.^2,5 These suppressive and traumatic stimuli drive increased genetic heterogeneity along with characteristic gene mutations in known tumor suppressor genes.⁶

A 62-year-old man presented with a nonhealing wound on the right hand of 5 years’ duration that had previously been attributed to a penetrating injury with a piece of copper from a refrigerant coolant system. The wound initially blistered and then eventually callused and developed areas of ulceration. The patient consulted multiple physicians for treatment of the intensely pruritic and ulcerated lesion. He received prescriptions for cephalexin, trimethoprim-sulfamethoxazole, doxycycline, clindamycin, and clobetasol cream, all of which offered minimal improvement. Home therapies including vitamin E and tea tree oil yielded no benefit. The lesion roughly quadrupled in size over the last 5 years.

Physical examination revealed a 7.5×4.2-cm ulcerated plaque with ragged borders and abundant central neoepithelialization on the right palmar surface (Figure 1). No gross motor or sensory defects were identified. There was no epitrochlear, axillary, cervical, or supraclavicular lymphadenopathy. A shave biopsy of the plaque’s edge was performed, which demonstrated a hyperplastic epidermis comprising atypical poroid cells with frequent mitoses, scant necrosis, and regular ductal structures confined to the epidermis (Figure 2). Immunohistochemical profiling results were positive for anticytokeratin (CAM 5.2) and Ber-EP4 (Figure 3). When evaluated in aggregate, these findings were consistent with porocarcinoma in situ.

The patient was referred to a surgical oncologist for evaluation. At that time, an exophytic mass had developed in the central lesion. Although no lymphadenopathy was identified upon examination, the patient had developed tremoring and a contracture deformity of the right hand. Extensive imaging and urgent surgical resection were recommended, but the patient did not wish to pursue these options, opting instead to continue home remedies. At a 15-month follow-up via telephone, the patient reported that the home therapy had failed and he had moved back to Vietnam. Partial limb amputation had been recommended by a local provider. Unfortunately, the patient was subsequently lost to follow-up, and his current status is unknown.

Porocarcinomas are rare tumors, comprising just 0.005% to 0.01% of all cutaneous epithelial tumors.^1,2,5 They affect men and women equally, with an average age at diagnosis of 60 to 70 years.^1,2 At least half of all porocarcinomas develop de novo, while 18% to 50% arise from the degeneration of an existing poroma.^2,3 Exposure to UV light and immunosuppression, particularly following organ transplantation, represent 2 commonly suspected catalysts for this malignant transformation.⁴ De novo porocarcinomas are most causatively linked to localized trauma from burns or radiation exposure.⁵ Gene mutations in classic tumor suppressor genes—tumor protein p53 (TP53), phosphatase and tensin homolog (PTEN), rearranged during transfection (RET), adenomatous polyposis coli (APC)—and increased genetic heterogeneity follow these stimuli.⁶

The morphologic presentation of porocarcinoma is highly variable and may manifest as papules, nodules, or plaques in various states of erosion, ulceration, or excoriation. Diagnoses of basal and squamous cell carcinoma, primary adnexal tumors, seborrheic keratosis, pyogenic granuloma, and melanoma must all be considered and methodically ruled out.⁷ Porocarcinomas may arise nearly anywhere on the body, with a particular predilection for the lower extremities (35%), head/neck (24%), and upper extremities (14%).^3,4 Primary lesions arising from the extremities, genitalia, or buttocks herald a higher risk for lymphatic invasion and distant metastasis, while head and neck tumors more commonly remain localized.⁸ Bleeding, ulceration, or rapid expansion of a preexisting poroma is suggestive of malignant transformation and may portend a more aggressive disease pattern.^2,9

Unequivocal diagnosis relies on histological and immunohistochemical studies due to the marked clinical variance of this neoplasm.⁷ An irregular histologic pattern of poromatous basaloid cells with ductal differentiation and cytologic atypia commonly are seen with porocarcinomas.^2,8 Nuclear pleomorphism with cellular necrosis, increased mitotic figures, and abortive ductal formation with a distinct lack of retraction around cellular aggregates often are found. Immunohistochemical staining is needed to confirm the primary tumor diagnosis. Histochemical stains commonly employed include carcinoembryonic antigen (CEA), cytokeratin AE1/AE3, epithelial membrane antigen, p53, p63, Ki67, and periodic acid-Schiff.¹⁰ The use of BerEP4 has been reported as efficacious in highlighting sweat structures, which can be particularly useful in cases when basal cell carcinoma is not in the histologic differential.¹¹ These staining profiles afford confirmation of ductal differentiation with CEA, epithelial membrane antigen, and BerEP4, while p63 and Ki67 are used as surrogates for primary cutaneous neoplasia and cell proliferation, respectively.^5,11 Porocarcinoma lesions may be most sensitive to CEA and most specific to CK19 (a component of cytokeratin AE1/AE3), though these findings have not been widely reproduced.⁷

The treatment and prognosis of porocarcinoma vary widely. Surgically excised lesions recur in roughly 20% of cases, though these rates likely include tumors that were incompletely resected in the primary attempt. Although wide local excision with an average 1-cm margin remains the most employed removal technique, Mohs micrographic surgery may more effectively limit recurrence and metastasis of localized disease.^7,8,12 Metastatic disease foretells a mortality rate of at least 65%, which is problematic in that 10% to 20% of patients have metastatic disease at the time of diagnosis and another 20% will show metastasis following primary tumor excision.^8,10 Neoplasms with high mitotic rates and depths greater than 7 mm should prompt thorough diagnostic imaging, such as positron emission tomography or magnetic resonance imaging. A sentinel lymph node biopsy should be strongly considered and discussed with the patient.¹⁰ Treatment options for nodal and distant metastases include a combination of localized surgery, lymphadenectomy, radiotherapy, and chemotherapeutic agents.^2,4,5 The response to systemic treatment and radiotherapy often is quite poor, though the use of combinations of docetaxel, paclitaxel, cetuximab, and immunotherapy have been efficacious in smaller studies.^8,10 The highest rates of morbidity and mortality are seen in patients with metastases on presentation or with localized tumors in the groin and buttocks.⁸

The diagnosis of porocarcinoma may be elusive due to its relatively rare occurrence. Therefore, it is critical to consider this neoplasm in high-risk sites in older patients who present with an evolving nodule or tumor on an extremity. Routine histology and astute histochemical profiling are necessary to exclude diseases that mimic porocarcinoma. Once diagnosis is confirmed, management with prompt excision and diagnostic imaging is recommended, including a lymph node biopsy if appropriate. Due to its high metastatic potential and associated morbidity and mortality, patients with porocarcinoma should be followed closely by a multidisciplinary care team.

To the Editor:

Porocarcinoma, or malignant poroma, is a rare adnexal malignancy of a predominantly glandular origin that comprises less than 0.01% of all cutaneous neoplasms.^1,2 Although exposure to UV radiation and immunosuppression have been implicated in the malignant degeneration of benign poromas into porocarcinomas, at least half of all malignant variants will arise de novo.^3,4 Patients present with an evolving nodule or plaque and often are in their seventh or eighth decade of life at the time of diagnosis.² Localized trauma from burns or radiation exposure has been causatively linked to de novo porocarcinoma formation.^2,5 These suppressive and traumatic stimuli drive increased genetic heterogeneity along with characteristic gene mutations in known tumor suppressor genes.⁶

A 62-year-old man presented with a nonhealing wound on the right hand of 5 years’ duration that had previously been attributed to a penetrating injury with a piece of copper from a refrigerant coolant system. The wound initially blistered and then eventually callused and developed areas of ulceration. The patient consulted multiple physicians for treatment of the intensely pruritic and ulcerated lesion. He received prescriptions for cephalexin, trimethoprim-sulfamethoxazole, doxycycline, clindamycin, and clobetasol cream, all of which offered minimal improvement. Home therapies including vitamin E and tea tree oil yielded no benefit. The lesion roughly quadrupled in size over the last 5 years.

Physical examination revealed a 7.5×4.2-cm ulcerated plaque with ragged borders and abundant central neoepithelialization on the right palmar surface (Figure 1). No gross motor or sensory defects were identified. There was no epitrochlear, axillary, cervical, or supraclavicular lymphadenopathy. A shave biopsy of the plaque’s edge was performed, which demonstrated a hyperplastic epidermis comprising atypical poroid cells with frequent mitoses, scant necrosis, and regular ductal structures confined to the epidermis (Figure 2). Immunohistochemical profiling results were positive for anticytokeratin (CAM 5.2) and Ber-EP4 (Figure 3). When evaluated in aggregate, these findings were consistent with porocarcinoma in situ.

The patient was referred to a surgical oncologist for evaluation. At that time, an exophytic mass had developed in the central lesion. Although no lymphadenopathy was identified upon examination, the patient had developed tremoring and a contracture deformity of the right hand. Extensive imaging and urgent surgical resection were recommended, but the patient did not wish to pursue these options, opting instead to continue home remedies. At a 15-month follow-up via telephone, the patient reported that the home therapy had failed and he had moved back to Vietnam. Partial limb amputation had been recommended by a local provider. Unfortunately, the patient was subsequently lost to follow-up, and his current status is unknown.

Porocarcinomas are rare tumors, comprising just 0.005% to 0.01% of all cutaneous epithelial tumors.^1,2,5 They affect men and women equally, with an average age at diagnosis of 60 to 70 years.^1,2 At least half of all porocarcinomas develop de novo, while 18% to 50% arise from the degeneration of an existing poroma.^2,3 Exposure to UV light and immunosuppression, particularly following organ transplantation, represent 2 commonly suspected catalysts for this malignant transformation.⁴ De novo porocarcinomas are most causatively linked to localized trauma from burns or radiation exposure.⁵ Gene mutations in classic tumor suppressor genes—tumor protein p53 (TP53), phosphatase and tensin homolog (PTEN), rearranged during transfection (RET), adenomatous polyposis coli (APC)—and increased genetic heterogeneity follow these stimuli.⁶

The morphologic presentation of porocarcinoma is highly variable and may manifest as papules, nodules, or plaques in various states of erosion, ulceration, or excoriation. Diagnoses of basal and squamous cell carcinoma, primary adnexal tumors, seborrheic keratosis, pyogenic granuloma, and melanoma must all be considered and methodically ruled out.⁷ Porocarcinomas may arise nearly anywhere on the body, with a particular predilection for the lower extremities (35%), head/neck (24%), and upper extremities (14%).^3,4 Primary lesions arising from the extremities, genitalia, or buttocks herald a higher risk for lymphatic invasion and distant metastasis, while head and neck tumors more commonly remain localized.⁸ Bleeding, ulceration, or rapid expansion of a preexisting poroma is suggestive of malignant transformation and may portend a more aggressive disease pattern.^2,9

Unequivocal diagnosis relies on histological and immunohistochemical studies due to the marked clinical variance of this neoplasm.⁷ An irregular histologic pattern of poromatous basaloid cells with ductal differentiation and cytologic atypia commonly are seen with porocarcinomas.^2,8 Nuclear pleomorphism with cellular necrosis, increased mitotic figures, and abortive ductal formation with a distinct lack of retraction around cellular aggregates often are found. Immunohistochemical staining is needed to confirm the primary tumor diagnosis. Histochemical stains commonly employed include carcinoembryonic antigen (CEA), cytokeratin AE1/AE3, epithelial membrane antigen, p53, p63, Ki67, and periodic acid-Schiff.¹⁰ The use of BerEP4 has been reported as efficacious in highlighting sweat structures, which can be particularly useful in cases when basal cell carcinoma is not in the histologic differential.¹¹ These staining profiles afford confirmation of ductal differentiation with CEA, epithelial membrane antigen, and BerEP4, while p63 and Ki67 are used as surrogates for primary cutaneous neoplasia and cell proliferation, respectively.^5,11 Porocarcinoma lesions may be most sensitive to CEA and most specific to CK19 (a component of cytokeratin AE1/AE3), though these findings have not been widely reproduced.⁷

The treatment and prognosis of porocarcinoma vary widely. Surgically excised lesions recur in roughly 20% of cases, though these rates likely include tumors that were incompletely resected in the primary attempt. Although wide local excision with an average 1-cm margin remains the most employed removal technique, Mohs micrographic surgery may more effectively limit recurrence and metastasis of localized disease.^7,8,12 Metastatic disease foretells a mortality rate of at least 65%, which is problematic in that 10% to 20% of patients have metastatic disease at the time of diagnosis and another 20% will show metastasis following primary tumor excision.^8,10 Neoplasms with high mitotic rates and depths greater than 7 mm should prompt thorough diagnostic imaging, such as positron emission tomography or magnetic resonance imaging. A sentinel lymph node biopsy should be strongly considered and discussed with the patient.¹⁰ Treatment options for nodal and distant metastases include a combination of localized surgery, lymphadenectomy, radiotherapy, and chemotherapeutic agents.^2,4,5 The response to systemic treatment and radiotherapy often is quite poor, though the use of combinations of docetaxel, paclitaxel, cetuximab, and immunotherapy have been efficacious in smaller studies.^8,10 The highest rates of morbidity and mortality are seen in patients with metastases on presentation or with localized tumors in the groin and buttocks.⁸

The diagnosis of porocarcinoma may be elusive due to its relatively rare occurrence. Therefore, it is critical to consider this neoplasm in high-risk sites in older patients who present with an evolving nodule or tumor on an extremity. Routine histology and astute histochemical profiling are necessary to exclude diseases that mimic porocarcinoma. Once diagnosis is confirmed, management with prompt excision and diagnostic imaging is recommended, including a lymph node biopsy if appropriate. Due to its high metastatic potential and associated morbidity and mortality, patients with porocarcinoma should be followed closely by a multidisciplinary care team.

The Diagnosis: Mpox (Monkeypox)

Tests for active herpes simplex virus (HHV), gonorrhea, chlamydia, HIV, and syphilis were negative. Swabs from the penile lesion demonstrated positivity for the West African clade of mpox (monkeypox) virus (MPXV) by polymerase chain reaction. The patient was treated supportively without the addition of antiviral therapy, and he experienced a complete recovery.

Mpox virus was first isolated in 1958 in a research facility and was named after the laboratory animals that were housed there. The first human documentation of the disease occurred in 1970, and it was first documented in the United States in 2003 in an infection that was traced to a shipment of small mammals from Ghana to Texas.¹ The disease has always been endemic to Africa; however, the incidence has been increasing.² A new MPXV outbreak was reported in many countries in early 2022, including the United States.¹

The MPXV is a double-stranded DNA virus of the genus Orthopoxvirus, and 2 genetic clades have been identified: clade I (formerly the Central African clade) and clade II (formerly the West African clade). The virus has the capability to infect many mammals; however, its host remains unidentified.¹ The exact mechanism of transmission from infected animals to humans largely is unknown; however, direct or indirect contact with infected animals likely is responsible. Human-to-human transmission can occur by many mechanisms including contact with large respiratory droplets, bodily fluids, and contaminated surfaces. The incubation period is 5 to 21 days, and the symptoms last 2 to 5 weeks.¹

The clinical manifestations of MPXV during the most recent outbreak differ from prior outbreaks. Patients are more likely to experience minimal to no systemic symptoms, and cutaneous lesions can be few and localized to a focal area, especially on the face and in the anogenital region,³ similar to the presentation in our patient (Figure 1). Cutaneous lesions of the most recent MPXV outbreak also include painless ulcerations similar to syphilitic chancres and lesions that are in various stages of healing.³ Lesions often begin as pseudopustules, which are firm white papules with or without a necrotic center that resemble pustules; unlike true pustules, there is no identifiable purulent material within it. Bacterial superinfection of the lesions is not uncommon.⁴ Over time, a secondary pustular eruption resembling folliculitis also may occur,4 as noted in our patient (Figure 2).

Although we did not have a biopsy to support the diagnosis of associated erythema multiforme (EM) in our patient, features supportive of this diagnosis included the classic clinical appearance of typical, well-defined, targetoid plaques with 3 distinct zones (Figure 3); the association with a known infection; the distribution on the arms with truncal sparing; and self-limited lesions. More than 90% of EM cases are associated with infection, with HHV representing the most common culprit⁵; therefore, the relationship with a different virus is not an unreasonable suggestion. Additionally, there have been rare reports of EM in association with MPXV.⁴

Histopathology of MPXV may have distinctive features. Lesions often demonstrate keratinocytic necrosis and basal layer vacuolization with an associated superficial and deep perivascular lymphohistiocytic infiltrate. When the morphology of the lesion is vesicular, histopathology reveals spongiosis and ballooning degeneration with epidermal necrosis. Viral inclusion bodies within keratinocytes may be identified.¹ Death rates from MPXV has been reported from 1% to 11%, with increased mortality among high-risk populations including children and immunocompromised individuals. Treatment of the disease largely consists of supportive care and management of any associated complications including bacterial infection, pneumonia, and encephalitis.¹

The differential diagnosis of MPXV includes other ulcerative lesions that can occur on the genital skin. Fixed drug eruptions often present on the penis,⁶ but there was no identifiable inciting drug in our patient. Herpes simplex virus infection was very high on the differential given our patient’s history of recurrent infections and association with a targetoid rash, but HHV type 1 and HHV type 2 testing of the lesion was negative. A syphilitic chancre also may present with the nontender genital ulceration⁷ that was seen in our patient, but serology did not support this diagnosis. Cutaneous Crohn disease also may manifest with genital ulceration even before a diagnosis of Crohn disease is made, but these lesions often present as linear knife-cut ulcerations of the anogenital region.⁸

Our case further supports a clinical presentation that diverges from the more traditional cases of MPXV. Additionally, associated EM may be a clue to infection, especially in cases of negative HHV and other sexually transmitted infection testing.

The Diagnosis: Mpox (Monkeypox)

Tests for active herpes simplex virus (HHV), gonorrhea, chlamydia, HIV, and syphilis were negative. Swabs from the penile lesion demonstrated positivity for the West African clade of mpox (monkeypox) virus (MPXV) by polymerase chain reaction. The patient was treated supportively without the addition of antiviral therapy, and he experienced a complete recovery.

Mpox virus was first isolated in 1958 in a research facility and was named after the laboratory animals that were housed there. The first human documentation of the disease occurred in 1970, and it was first documented in the United States in 2003 in an infection that was traced to a shipment of small mammals from Ghana to Texas.¹ The disease has always been endemic to Africa; however, the incidence has been increasing.² A new MPXV outbreak was reported in many countries in early 2022, including the United States.¹

The MPXV is a double-stranded DNA virus of the genus Orthopoxvirus, and 2 genetic clades have been identified: clade I (formerly the Central African clade) and clade II (formerly the West African clade). The virus has the capability to infect many mammals; however, its host remains unidentified.¹ The exact mechanism of transmission from infected animals to humans largely is unknown; however, direct or indirect contact with infected animals likely is responsible. Human-to-human transmission can occur by many mechanisms including contact with large respiratory droplets, bodily fluids, and contaminated surfaces. The incubation period is 5 to 21 days, and the symptoms last 2 to 5 weeks.¹

The clinical manifestations of MPXV during the most recent outbreak differ from prior outbreaks. Patients are more likely to experience minimal to no systemic symptoms, and cutaneous lesions can be few and localized to a focal area, especially on the face and in the anogenital region,³ similar to the presentation in our patient (Figure 1). Cutaneous lesions of the most recent MPXV outbreak also include painless ulcerations similar to syphilitic chancres and lesions that are in various stages of healing.³ Lesions often begin as pseudopustules, which are firm white papules with or without a necrotic center that resemble pustules; unlike true pustules, there is no identifiable purulent material within it. Bacterial superinfection of the lesions is not uncommon.⁴ Over time, a secondary pustular eruption resembling folliculitis also may occur,4 as noted in our patient (Figure 2).

Although we did not have a biopsy to support the diagnosis of associated erythema multiforme (EM) in our patient, features supportive of this diagnosis included the classic clinical appearance of typical, well-defined, targetoid plaques with 3 distinct zones (Figure 3); the association with a known infection; the distribution on the arms with truncal sparing; and self-limited lesions. More than 90% of EM cases are associated with infection, with HHV representing the most common culprit⁵; therefore, the relationship with a different virus is not an unreasonable suggestion. Additionally, there have been rare reports of EM in association with MPXV.⁴

Histopathology of MPXV may have distinctive features. Lesions often demonstrate keratinocytic necrosis and basal layer vacuolization with an associated superficial and deep perivascular lymphohistiocytic infiltrate. When the morphology of the lesion is vesicular, histopathology reveals spongiosis and ballooning degeneration with epidermal necrosis. Viral inclusion bodies within keratinocytes may be identified.¹ Death rates from MPXV has been reported from 1% to 11%, with increased mortality among high-risk populations including children and immunocompromised individuals. Treatment of the disease largely consists of supportive care and management of any associated complications including bacterial infection, pneumonia, and encephalitis.¹

The differential diagnosis of MPXV includes other ulcerative lesions that can occur on the genital skin. Fixed drug eruptions often present on the penis,⁶ but there was no identifiable inciting drug in our patient. Herpes simplex virus infection was very high on the differential given our patient’s history of recurrent infections and association with a targetoid rash, but HHV type 1 and HHV type 2 testing of the lesion was negative. A syphilitic chancre also may present with the nontender genital ulceration⁷ that was seen in our patient, but serology did not support this diagnosis. Cutaneous Crohn disease also may manifest with genital ulceration even before a diagnosis of Crohn disease is made, but these lesions often present as linear knife-cut ulcerations of the anogenital region.⁸

Our case further supports a clinical presentation that diverges from the more traditional cases of MPXV. Additionally, associated EM may be a clue to infection, especially in cases of negative HHV and other sexually transmitted infection testing.

The Diagnosis: Mpox (Monkeypox)

Tests for active herpes simplex virus (HHV), gonorrhea, chlamydia, HIV, and syphilis were negative. Swabs from the penile lesion demonstrated positivity for the West African clade of mpox (monkeypox) virus (MPXV) by polymerase chain reaction. The patient was treated supportively without the addition of antiviral therapy, and he experienced a complete recovery.

Mpox virus was first isolated in 1958 in a research facility and was named after the laboratory animals that were housed there. The first human documentation of the disease occurred in 1970, and it was first documented in the United States in 2003 in an infection that was traced to a shipment of small mammals from Ghana to Texas.¹ The disease has always been endemic to Africa; however, the incidence has been increasing.² A new MPXV outbreak was reported in many countries in early 2022, including the United States.¹

The MPXV is a double-stranded DNA virus of the genus Orthopoxvirus, and 2 genetic clades have been identified: clade I (formerly the Central African clade) and clade II (formerly the West African clade). The virus has the capability to infect many mammals; however, its host remains unidentified.¹ The exact mechanism of transmission from infected animals to humans largely is unknown; however, direct or indirect contact with infected animals likely is responsible. Human-to-human transmission can occur by many mechanisms including contact with large respiratory droplets, bodily fluids, and contaminated surfaces. The incubation period is 5 to 21 days, and the symptoms last 2 to 5 weeks.¹

The clinical manifestations of MPXV during the most recent outbreak differ from prior outbreaks. Patients are more likely to experience minimal to no systemic symptoms, and cutaneous lesions can be few and localized to a focal area, especially on the face and in the anogenital region,³ similar to the presentation in our patient (Figure 1). Cutaneous lesions of the most recent MPXV outbreak also include painless ulcerations similar to syphilitic chancres and lesions that are in various stages of healing.³ Lesions often begin as pseudopustules, which are firm white papules with or without a necrotic center that resemble pustules; unlike true pustules, there is no identifiable purulent material within it. Bacterial superinfection of the lesions is not uncommon.⁴ Over time, a secondary pustular eruption resembling folliculitis also may occur,4 as noted in our patient (Figure 2).

Although we did not have a biopsy to support the diagnosis of associated erythema multiforme (EM) in our patient, features supportive of this diagnosis included the classic clinical appearance of typical, well-defined, targetoid plaques with 3 distinct zones (Figure 3); the association with a known infection; the distribution on the arms with truncal sparing; and self-limited lesions. More than 90% of EM cases are associated with infection, with HHV representing the most common culprit⁵; therefore, the relationship with a different virus is not an unreasonable suggestion. Additionally, there have been rare reports of EM in association with MPXV.⁴

Histopathology of MPXV may have distinctive features. Lesions often demonstrate keratinocytic necrosis and basal layer vacuolization with an associated superficial and deep perivascular lymphohistiocytic infiltrate. When the morphology of the lesion is vesicular, histopathology reveals spongiosis and ballooning degeneration with epidermal necrosis. Viral inclusion bodies within keratinocytes may be identified.¹ Death rates from MPXV has been reported from 1% to 11%, with increased mortality among high-risk populations including children and immunocompromised individuals. Treatment of the disease largely consists of supportive care and management of any associated complications including bacterial infection, pneumonia, and encephalitis.¹

The differential diagnosis of MPXV includes other ulcerative lesions that can occur on the genital skin. Fixed drug eruptions often present on the penis,⁶ but there was no identifiable inciting drug in our patient. Herpes simplex virus infection was very high on the differential given our patient’s history of recurrent infections and association with a targetoid rash, but HHV type 1 and HHV type 2 testing of the lesion was negative. A syphilitic chancre also may present with the nontender genital ulceration⁷ that was seen in our patient, but serology did not support this diagnosis. Cutaneous Crohn disease also may manifest with genital ulceration even before a diagnosis of Crohn disease is made, but these lesions often present as linear knife-cut ulcerations of the anogenital region.⁸

Our case further supports a clinical presentation that diverges from the more traditional cases of MPXV. Additionally, associated EM may be a clue to infection, especially in cases of negative HHV and other sexually transmitted infection testing.

Hidradenitis suppurativa (HS), a chronic inflammatory disease that is characterized by tender inflamed nodules of the skin and subcutaneous tissue, disproportionately affects postpubertal females as well as Black/African American individuals. The nodules can rupture, form sinus tracts, and scar. ¹ Hidradenitis suppurativa has been associated with cardiovascular disease, type 2 diabetes mellitus, polycystic ovary syndrome, depression, suicide, and substance use disorders. Because of the symptom burden and associated conditions, HS can be a painful and distressing disease that substantially impairs the quality of life for individuals with this condition. ²

Pain is a commonly reported symptom in HS that often goes untreated. Furthermore, HS-related pain is complex due to the involvement of different pain types that require various treatment modalities.³ According to Savage et al,⁴ recognizing whether HS-related pain is acute, chronic, neuropathic, or nociceptive is vital in establishing a framework for an effective pain management scheme. Currently, such established multimodal pain management strategies in dermatology do not exist. In 2021, dermatology-specific pain management strategies proposed the use of a multimodal regimen to address the multifaceted nature of HS-related pain.⁴ However, these strategies failed to recognize the systemic racial and ethnic biases in the US health care system that undermine pain management care for minority groups.^5,6 One approach to combatting racial disparities in pain management is determining average pain levels across racial groups.⁷ This study sought to compare HS-related pain scores by racial groups. Furthermore, we assessed differences in perception of patients’ respective pain management regimens by race. We hypothesized that the average HS-related pain intensities and pain management would differ between self-reported racial groups.

Methods  

This cross-sectional study took place over 5 months (August through December 2021). A survey was emailed to 2198 adult patients with HS in the University of Alabama Health System. The survey consisted of demographic and general questions about a patient’s HS. Pain scores were captured using the numeric rating scale (NRS), a measurement tool for pain intensity on a scale from 0 to 10. ⁸ Age at diagnosis, gender, education level, household income, total body areas affected by HS, disease severity (categorized as mild, moderate, and severe), comorbidities including mood disorders, tobacco use, and HS and HS-related pain medication regimens also were collected. Additionally, participants were asked about their level of agreement with the following statements: “I am satisfied with how my pain related to HS is being managed by my doctors” and “My pain related to HS is under control.” The level of agreement was measured using a 5-point Likert scale, with responses ranging from strongly disagree to strongly agree. All data included in the analysis were self-reported. The study received institutional review board approval for the University of Alabama at Birmingham.

Statistical Analysis—Descriptive statistics were used to assess statistical differences in patient characteristics of Black/African American participants compared to other participants, including White, Asian, and Hispanic/Latino participants. Thirteen participants were excluded from the final analysis: 2 participants were missing data, and 11 biracial participants were excluded due to overlapping White and Black/African American races that may have confounded the analysis. Categorical variables were reported as frequencies and percentages, and χ² and Fisher exact tests, when necessary, were used to test for statistically significant differences. Continuous variables were summarized with means and standard deviations, and a t test was used for statistically significant differences.

Logistic regression was performed to assess the relationship between race and pain after adjusting for confounding variables such as obesity, current tobacco use, self-reported HS severity, and the presence of comorbidities. A total of 204 patient records were included in the analysis, of which 70 (34.3%) had a pain score of 8 or higher, which indicates very severe pain intensity levels on the NRS,⁸ and were selected as a cut point based on the distribution of responses. For this cross-sectional cohort, our approach was to compare characteristics of those classified with a top score of 8 or higher (n=70) vs a top score of 0 to 7 (n=134)(cases vs noncases). Statistical analyses were performed using JMP Pro 16 (JMP Statistical Discovery LLC) at an α=.05 significance level; logistic regression was performed using SPSS Statistics (IBM). For the logistic regression, we grouped patient race into 2 categories: Black/African American and Other, which included White, Asian, and Hispanic/Latino participants.

Crude and adjusted multivariable logistic regression analyses were used to calculate prevalence odds ratios with 95% confidence intervals. Covariate inclusion in the multivariable logistic regression was based on a priori hypothesis/knowledge and was meant to estimate the independent effect of race after adjustment for income, HS severity, and history of prescription pain medication use. Other variables, including tobacco use, obesity, mood disorders, and current HS treatments, were all individually tested in the multivariate analysis and did not significantly impact the odds ratio for high pain. Statistical adjustment slightly decreased (19%) the magnitude between crude and adjusted prevalence odds ratios for the association between Black/African American race and high pain score.

Results  

Survey Demographics —The final analysis included 204 survey respondents. Most respondents were Black/African American (58.82%), and nearly all were female (89.71%)(Table 1). The mean age (SD) of respondents was 37.37 (11.29) years (range, 19-70 years). Many respondents reported having completed some college (36.27%) or receiving a bachelor’s degree (19.12%). Of patients who were not Black/African American, 10.71% had higher than a master’s degree, whereas no Black/African American patients held a degree higher than a master’s ( P = .0052). Additionally, more Black/African American respondents (35.83%) reported an annual household income level of less than $25,000 compared with respondents who were not Black/African American (19.05%, P = .0001). Most respondents rated the severity of their HS as moderate or severe (46.57% and 41.67%, respectively), and there was no significant difference in reported severity of HS between racial groups ( P = .5395).

Pain Scores—As documented in the Methods, respondents were asked to rate their HS-related pain intensity from 0 to 10 using the NRS. The average pain score (SD)—the level of pain intensity over the prior month—was 6.39 (2.56)(range, 0–10). The mean pain score (SD) at the time of the survey was 3.61 (2.98)(range, 0–10)(Table 1). These data revealed that Black/African American patients had a significantly higher average pain score (SD) than patients with HS who were not Black/African American (7.08 [2.49] and 5.40 [2.35], respectively; P<.0001). After adjustment with multivariable logistical regression, Black/African American patients had 4-fold increased odds for very severe levels of pain (score of ≥8) compared with patients who were not Black/African American.

Pain Management—Although pain scores were higher for Black/African American patients with HS, there was no significant difference in the perception of pain control between racial groups (P=.0761). Additionally, we found low income (adjusted prevalence odds ratio [POR], 0.22; 95% CI, 0.05-0.91), a history of prescription pain medication use (adjusted POR, 2.25; 95% CI, 1.13-4.51), and HS severity (adjusted POR, 4.40; 95% CI, 1.11-17.36) all to be independent risk factors contributing to higher pain scores in patients with HS (Table 2). Lastly, we noted current or reported history of pain medication use was significantly correlated with higher pain scores (P=.0280 and P=.0213, respectively).

Satisfaction With Pain Management—The level of satisfaction with physician management of HS-related pain was significantly different between Black/African American patients and those who were not Black/African American (P=.0129). Of those who identified as Black/African American, 26.7% (n=32) strongly disagreed with the statement, “I am satisfied with how my pain related to HS is being managed by my doctors,” whereas only 15.5% (n=13) of patients who were not Black/African American strongly disagreed. 

Comment

There is no cure for HS, and a large focus of treatment is pain management. Because racial disparities in the treatment of chronic pain will affect those with HS, we conducted a cross-sectional analysis of pain and pain management among HS patients. We found that Black/African American patients with HS have higher average pain scores than those who are not Black/African American and were 4 times more likely to experience very severe pain. Prior studies have established that patients with HS often report higher pain levels than patients with other chronic inflammatory skin conditions, ^7,8 and our study identified racial disparities in HS-related pain management.

Measuring pain is challenging because of its multidimensional and subjective nature, making it essential to consider underlying causes and patients’ emotional responses to pain.⁹ By adjusting for confounding factors that may influence pain, such as mood disorders, disease severity, comorbidities, and medication use, we were able to gain better insight into fundamental differences in average pain intensity levels among racial groups and assess what factors may be contributing to a patient’s pain perception. Our study determined that lower income levels, higher HS disease severity, and a history of prescription pain medication use were all independent risk factors for high pain. Of note, obesity, tobacco use, and mood disorders such as anxiety and depression did not significantly differ between racial groups or increase the odds of high pain between racial groups identified.

With low income being an independent risk factor for high pain, we must consider the social determinants of health and how they may influence the pain experience in HS. We speculate that low income may be associated with other social determinants of health for the patients assessed in this study, such as lack of social and community support or limited health care access that contribute to worse health outcomes.^10,11 In addition, low income contributes to limited access to medical care or treatments¹²; without access to effective HS management, lower-income patients may be at risk for higher disease severity and thus higher pain levels. However, economic stability is only a part of the whole picture; therefore, assessing the other social determinants of health in patients with HS may lead to better health outcomes and quality of life.

Another identified risk factor for high pain was a reported history of prescription pain medication use. This finding suggests that patients with moderate to severe pain likely have required stronger analgesic medications in the past. We further speculate that high pain levels in patients who have received prescription pain medications indicate either undertreatment, mistreatment, or recalcitrant pain. More research is needed to assess the relationship between HS-related pain intensity, analgesic medications, and providers who manage HS-related pain.

We also found that Black/African American patients with HS had a significantly higher dissatisfaction with their physician’s management of their pain, which could be attributable to several factors, including biological differences in medication metabolism (in which the patient has medication-resistant HS), undertreatment of pain, and/or poor doctor-patient relations. These reasons coincide with other diseases where health disparities are found.^13-15 Recognizing these factors will be key to dismantling the disparities in HS that are noted within this study. The limitations of this work include the cross-sectional study design and its inability to evaluate causal factors of high pain levels across racial groups, the NRS lack of insight on pain chronicity or pain experience,⁷ the lack of provider or institution perspectives, and self-reported data. Additionally, only patients with email access were included, which may have excluded vulnerable populations with more pain associated with their HS.

Our findings highlight an area for further investigation to assess why these racial differences exist in HS-related pain. The results also emphasize the need for research evaluating whether systemic or health care provider biases contribute to racial differences in HS-related pain management.

Acknowledgment — Dr. Weir was supported by the Predoctoral Clinical/Translational Research Program (TL1), a National Institutes of Health Ruth L. Kirschstein National Research Service Award (NRSA), through the University of Alabama at Birmingham (UAB) Center for Clinical and Translational Science (CCTS).  

Hidradenitis suppurativa (HS), a chronic inflammatory disease that is characterized by tender inflamed nodules of the skin and subcutaneous tissue, disproportionately affects postpubertal females as well as Black/African American individuals. The nodules can rupture, form sinus tracts, and scar. ¹ Hidradenitis suppurativa has been associated with cardiovascular disease, type 2 diabetes mellitus, polycystic ovary syndrome, depression, suicide, and substance use disorders. Because of the symptom burden and associated conditions, HS can be a painful and distressing disease that substantially impairs the quality of life for individuals with this condition. ²

Pain is a commonly reported symptom in HS that often goes untreated. Furthermore, HS-related pain is complex due to the involvement of different pain types that require various treatment modalities.³ According to Savage et al,⁴ recognizing whether HS-related pain is acute, chronic, neuropathic, or nociceptive is vital in establishing a framework for an effective pain management scheme. Currently, such established multimodal pain management strategies in dermatology do not exist. In 2021, dermatology-specific pain management strategies proposed the use of a multimodal regimen to address the multifaceted nature of HS-related pain.⁴ However, these strategies failed to recognize the systemic racial and ethnic biases in the US health care system that undermine pain management care for minority groups.^5,6 One approach to combatting racial disparities in pain management is determining average pain levels across racial groups.⁷ This study sought to compare HS-related pain scores by racial groups. Furthermore, we assessed differences in perception of patients’ respective pain management regimens by race. We hypothesized that the average HS-related pain intensities and pain management would differ between self-reported racial groups.

Methods  

This cross-sectional study took place over 5 months (August through December 2021). A survey was emailed to 2198 adult patients with HS in the University of Alabama Health System. The survey consisted of demographic and general questions about a patient’s HS. Pain scores were captured using the numeric rating scale (NRS), a measurement tool for pain intensity on a scale from 0 to 10. ⁸ Age at diagnosis, gender, education level, household income, total body areas affected by HS, disease severity (categorized as mild, moderate, and severe), comorbidities including mood disorders, tobacco use, and HS and HS-related pain medication regimens also were collected. Additionally, participants were asked about their level of agreement with the following statements: “I am satisfied with how my pain related to HS is being managed by my doctors” and “My pain related to HS is under control.” The level of agreement was measured using a 5-point Likert scale, with responses ranging from strongly disagree to strongly agree. All data included in the analysis were self-reported. The study received institutional review board approval for the University of Alabama at Birmingham.

Statistical Analysis—Descriptive statistics were used to assess statistical differences in patient characteristics of Black/African American participants compared to other participants, including White, Asian, and Hispanic/Latino participants. Thirteen participants were excluded from the final analysis: 2 participants were missing data, and 11 biracial participants were excluded due to overlapping White and Black/African American races that may have confounded the analysis. Categorical variables were reported as frequencies and percentages, and χ² and Fisher exact tests, when necessary, were used to test for statistically significant differences. Continuous variables were summarized with means and standard deviations, and a t test was used for statistically significant differences.

Logistic regression was performed to assess the relationship between race and pain after adjusting for confounding variables such as obesity, current tobacco use, self-reported HS severity, and the presence of comorbidities. A total of 204 patient records were included in the analysis, of which 70 (34.3%) had a pain score of 8 or higher, which indicates very severe pain intensity levels on the NRS,⁸ and were selected as a cut point based on the distribution of responses. For this cross-sectional cohort, our approach was to compare characteristics of those classified with a top score of 8 or higher (n=70) vs a top score of 0 to 7 (n=134)(cases vs noncases). Statistical analyses were performed using JMP Pro 16 (JMP Statistical Discovery LLC) at an α=.05 significance level; logistic regression was performed using SPSS Statistics (IBM). For the logistic regression, we grouped patient race into 2 categories: Black/African American and Other, which included White, Asian, and Hispanic/Latino participants.

Crude and adjusted multivariable logistic regression analyses were used to calculate prevalence odds ratios with 95% confidence intervals. Covariate inclusion in the multivariable logistic regression was based on a priori hypothesis/knowledge and was meant to estimate the independent effect of race after adjustment for income, HS severity, and history of prescription pain medication use. Other variables, including tobacco use, obesity, mood disorders, and current HS treatments, were all individually tested in the multivariate analysis and did not significantly impact the odds ratio for high pain. Statistical adjustment slightly decreased (19%) the magnitude between crude and adjusted prevalence odds ratios for the association between Black/African American race and high pain score.

Results  

Survey Demographics —The final analysis included 204 survey respondents. Most respondents were Black/African American (58.82%), and nearly all were female (89.71%)(Table 1). The mean age (SD) of respondents was 37.37 (11.29) years (range, 19-70 years). Many respondents reported having completed some college (36.27%) or receiving a bachelor’s degree (19.12%). Of patients who were not Black/African American, 10.71% had higher than a master’s degree, whereas no Black/African American patients held a degree higher than a master’s ( P = .0052). Additionally, more Black/African American respondents (35.83%) reported an annual household income level of less than $25,000 compared with respondents who were not Black/African American (19.05%, P = .0001). Most respondents rated the severity of their HS as moderate or severe (46.57% and 41.67%, respectively), and there was no significant difference in reported severity of HS between racial groups ( P = .5395).

Pain Scores—As documented in the Methods, respondents were asked to rate their HS-related pain intensity from 0 to 10 using the NRS. The average pain score (SD)—the level of pain intensity over the prior month—was 6.39 (2.56)(range, 0–10). The mean pain score (SD) at the time of the survey was 3.61 (2.98)(range, 0–10)(Table 1). These data revealed that Black/African American patients had a significantly higher average pain score (SD) than patients with HS who were not Black/African American (7.08 [2.49] and 5.40 [2.35], respectively; P<.0001). After adjustment with multivariable logistical regression, Black/African American patients had 4-fold increased odds for very severe levels of pain (score of ≥8) compared with patients who were not Black/African American.

Pain Management—Although pain scores were higher for Black/African American patients with HS, there was no significant difference in the perception of pain control between racial groups (P=.0761). Additionally, we found low income (adjusted prevalence odds ratio [POR], 0.22; 95% CI, 0.05-0.91), a history of prescription pain medication use (adjusted POR, 2.25; 95% CI, 1.13-4.51), and HS severity (adjusted POR, 4.40; 95% CI, 1.11-17.36) all to be independent risk factors contributing to higher pain scores in patients with HS (Table 2). Lastly, we noted current or reported history of pain medication use was significantly correlated with higher pain scores (P=.0280 and P=.0213, respectively).

Satisfaction With Pain Management—The level of satisfaction with physician management of HS-related pain was significantly different between Black/African American patients and those who were not Black/African American (P=.0129). Of those who identified as Black/African American, 26.7% (n=32) strongly disagreed with the statement, “I am satisfied with how my pain related to HS is being managed by my doctors,” whereas only 15.5% (n=13) of patients who were not Black/African American strongly disagreed. 

Comment

There is no cure for HS, and a large focus of treatment is pain management. Because racial disparities in the treatment of chronic pain will affect those with HS, we conducted a cross-sectional analysis of pain and pain management among HS patients. We found that Black/African American patients with HS have higher average pain scores than those who are not Black/African American and were 4 times more likely to experience very severe pain. Prior studies have established that patients with HS often report higher pain levels than patients with other chronic inflammatory skin conditions, ^7,8 and our study identified racial disparities in HS-related pain management.

Measuring pain is challenging because of its multidimensional and subjective nature, making it essential to consider underlying causes and patients’ emotional responses to pain.⁹ By adjusting for confounding factors that may influence pain, such as mood disorders, disease severity, comorbidities, and medication use, we were able to gain better insight into fundamental differences in average pain intensity levels among racial groups and assess what factors may be contributing to a patient’s pain perception. Our study determined that lower income levels, higher HS disease severity, and a history of prescription pain medication use were all independent risk factors for high pain. Of note, obesity, tobacco use, and mood disorders such as anxiety and depression did not significantly differ between racial groups or increase the odds of high pain between racial groups identified.

With low income being an independent risk factor for high pain, we must consider the social determinants of health and how they may influence the pain experience in HS. We speculate that low income may be associated with other social determinants of health for the patients assessed in this study, such as lack of social and community support or limited health care access that contribute to worse health outcomes.^10,11 In addition, low income contributes to limited access to medical care or treatments¹²; without access to effective HS management, lower-income patients may be at risk for higher disease severity and thus higher pain levels. However, economic stability is only a part of the whole picture; therefore, assessing the other social determinants of health in patients with HS may lead to better health outcomes and quality of life.

Another identified risk factor for high pain was a reported history of prescription pain medication use. This finding suggests that patients with moderate to severe pain likely have required stronger analgesic medications in the past. We further speculate that high pain levels in patients who have received prescription pain medications indicate either undertreatment, mistreatment, or recalcitrant pain. More research is needed to assess the relationship between HS-related pain intensity, analgesic medications, and providers who manage HS-related pain.

We also found that Black/African American patients with HS had a significantly higher dissatisfaction with their physician’s management of their pain, which could be attributable to several factors, including biological differences in medication metabolism (in which the patient has medication-resistant HS), undertreatment of pain, and/or poor doctor-patient relations. These reasons coincide with other diseases where health disparities are found.^13-15 Recognizing these factors will be key to dismantling the disparities in HS that are noted within this study. The limitations of this work include the cross-sectional study design and its inability to evaluate causal factors of high pain levels across racial groups, the NRS lack of insight on pain chronicity or pain experience,⁷ the lack of provider or institution perspectives, and self-reported data. Additionally, only patients with email access were included, which may have excluded vulnerable populations with more pain associated with their HS.

Our findings highlight an area for further investigation to assess why these racial differences exist in HS-related pain. The results also emphasize the need for research evaluating whether systemic or health care provider biases contribute to racial differences in HS-related pain management.

Acknowledgment — Dr. Weir was supported by the Predoctoral Clinical/Translational Research Program (TL1), a National Institutes of Health Ruth L. Kirschstein National Research Service Award (NRSA), through the University of Alabama at Birmingham (UAB) Center for Clinical and Translational Science (CCTS).  

Hidradenitis suppurativa (HS), a chronic inflammatory disease that is characterized by tender inflamed nodules of the skin and subcutaneous tissue, disproportionately affects postpubertal females as well as Black/African American individuals. The nodules can rupture, form sinus tracts, and scar. ¹ Hidradenitis suppurativa has been associated with cardiovascular disease, type 2 diabetes mellitus, polycystic ovary syndrome, depression, suicide, and substance use disorders. Because of the symptom burden and associated conditions, HS can be a painful and distressing disease that substantially impairs the quality of life for individuals with this condition. ²

Pain is a commonly reported symptom in HS that often goes untreated. Furthermore, HS-related pain is complex due to the involvement of different pain types that require various treatment modalities.³ According to Savage et al,⁴ recognizing whether HS-related pain is acute, chronic, neuropathic, or nociceptive is vital in establishing a framework for an effective pain management scheme. Currently, such established multimodal pain management strategies in dermatology do not exist. In 2021, dermatology-specific pain management strategies proposed the use of a multimodal regimen to address the multifaceted nature of HS-related pain.⁴ However, these strategies failed to recognize the systemic racial and ethnic biases in the US health care system that undermine pain management care for minority groups.^5,6 One approach to combatting racial disparities in pain management is determining average pain levels across racial groups.⁷ This study sought to compare HS-related pain scores by racial groups. Furthermore, we assessed differences in perception of patients’ respective pain management regimens by race. We hypothesized that the average HS-related pain intensities and pain management would differ between self-reported racial groups.

Methods  

This cross-sectional study took place over 5 months (August through December 2021). A survey was emailed to 2198 adult patients with HS in the University of Alabama Health System. The survey consisted of demographic and general questions about a patient’s HS. Pain scores were captured using the numeric rating scale (NRS), a measurement tool for pain intensity on a scale from 0 to 10. ⁸ Age at diagnosis, gender, education level, household income, total body areas affected by HS, disease severity (categorized as mild, moderate, and severe), comorbidities including mood disorders, tobacco use, and HS and HS-related pain medication regimens also were collected. Additionally, participants were asked about their level of agreement with the following statements: “I am satisfied with how my pain related to HS is being managed by my doctors” and “My pain related to HS is under control.” The level of agreement was measured using a 5-point Likert scale, with responses ranging from strongly disagree to strongly agree. All data included in the analysis were self-reported. The study received institutional review board approval for the University of Alabama at Birmingham.

Statistical Analysis—Descriptive statistics were used to assess statistical differences in patient characteristics of Black/African American participants compared to other participants, including White, Asian, and Hispanic/Latino participants. Thirteen participants were excluded from the final analysis: 2 participants were missing data, and 11 biracial participants were excluded due to overlapping White and Black/African American races that may have confounded the analysis. Categorical variables were reported as frequencies and percentages, and χ² and Fisher exact tests, when necessary, were used to test for statistically significant differences. Continuous variables were summarized with means and standard deviations, and a t test was used for statistically significant differences.

Logistic regression was performed to assess the relationship between race and pain after adjusting for confounding variables such as obesity, current tobacco use, self-reported HS severity, and the presence of comorbidities. A total of 204 patient records were included in the analysis, of which 70 (34.3%) had a pain score of 8 or higher, which indicates very severe pain intensity levels on the NRS,⁸ and were selected as a cut point based on the distribution of responses. For this cross-sectional cohort, our approach was to compare characteristics of those classified with a top score of 8 or higher (n=70) vs a top score of 0 to 7 (n=134)(cases vs noncases). Statistical analyses were performed using JMP Pro 16 (JMP Statistical Discovery LLC) at an α=.05 significance level; logistic regression was performed using SPSS Statistics (IBM). For the logistic regression, we grouped patient race into 2 categories: Black/African American and Other, which included White, Asian, and Hispanic/Latino participants.

Crude and adjusted multivariable logistic regression analyses were used to calculate prevalence odds ratios with 95% confidence intervals. Covariate inclusion in the multivariable logistic regression was based on a priori hypothesis/knowledge and was meant to estimate the independent effect of race after adjustment for income, HS severity, and history of prescription pain medication use. Other variables, including tobacco use, obesity, mood disorders, and current HS treatments, were all individually tested in the multivariate analysis and did not significantly impact the odds ratio for high pain. Statistical adjustment slightly decreased (19%) the magnitude between crude and adjusted prevalence odds ratios for the association between Black/African American race and high pain score.

Results  

Survey Demographics —The final analysis included 204 survey respondents. Most respondents were Black/African American (58.82%), and nearly all were female (89.71%)(Table 1). The mean age (SD) of respondents was 37.37 (11.29) years (range, 19-70 years). Many respondents reported having completed some college (36.27%) or receiving a bachelor’s degree (19.12%). Of patients who were not Black/African American, 10.71% had higher than a master’s degree, whereas no Black/African American patients held a degree higher than a master’s ( P = .0052). Additionally, more Black/African American respondents (35.83%) reported an annual household income level of less than $25,000 compared with respondents who were not Black/African American (19.05%, P = .0001). Most respondents rated the severity of their HS as moderate or severe (46.57% and 41.67%, respectively), and there was no significant difference in reported severity of HS between racial groups ( P = .5395).

Pain Scores—As documented in the Methods, respondents were asked to rate their HS-related pain intensity from 0 to 10 using the NRS. The average pain score (SD)—the level of pain intensity over the prior month—was 6.39 (2.56)(range, 0–10). The mean pain score (SD) at the time of the survey was 3.61 (2.98)(range, 0–10)(Table 1). These data revealed that Black/African American patients had a significantly higher average pain score (SD) than patients with HS who were not Black/African American (7.08 [2.49] and 5.40 [2.35], respectively; P<.0001). After adjustment with multivariable logistical regression, Black/African American patients had 4-fold increased odds for very severe levels of pain (score of ≥8) compared with patients who were not Black/African American.

Pain Management—Although pain scores were higher for Black/African American patients with HS, there was no significant difference in the perception of pain control between racial groups (P=.0761). Additionally, we found low income (adjusted prevalence odds ratio [POR], 0.22; 95% CI, 0.05-0.91), a history of prescription pain medication use (adjusted POR, 2.25; 95% CI, 1.13-4.51), and HS severity (adjusted POR, 4.40; 95% CI, 1.11-17.36) all to be independent risk factors contributing to higher pain scores in patients with HS (Table 2). Lastly, we noted current or reported history of pain medication use was significantly correlated with higher pain scores (P=.0280 and P=.0213, respectively).

Satisfaction With Pain Management—The level of satisfaction with physician management of HS-related pain was significantly different between Black/African American patients and those who were not Black/African American (P=.0129). Of those who identified as Black/African American, 26.7% (n=32) strongly disagreed with the statement, “I am satisfied with how my pain related to HS is being managed by my doctors,” whereas only 15.5% (n=13) of patients who were not Black/African American strongly disagreed. 

Comment

There is no cure for HS, and a large focus of treatment is pain management. Because racial disparities in the treatment of chronic pain will affect those with HS, we conducted a cross-sectional analysis of pain and pain management among HS patients. We found that Black/African American patients with HS have higher average pain scores than those who are not Black/African American and were 4 times more likely to experience very severe pain. Prior studies have established that patients with HS often report higher pain levels than patients with other chronic inflammatory skin conditions, ^7,8 and our study identified racial disparities in HS-related pain management.

Measuring pain is challenging because of its multidimensional and subjective nature, making it essential to consider underlying causes and patients’ emotional responses to pain.⁹ By adjusting for confounding factors that may influence pain, such as mood disorders, disease severity, comorbidities, and medication use, we were able to gain better insight into fundamental differences in average pain intensity levels among racial groups and assess what factors may be contributing to a patient’s pain perception. Our study determined that lower income levels, higher HS disease severity, and a history of prescription pain medication use were all independent risk factors for high pain. Of note, obesity, tobacco use, and mood disorders such as anxiety and depression did not significantly differ between racial groups or increase the odds of high pain between racial groups identified.

With low income being an independent risk factor for high pain, we must consider the social determinants of health and how they may influence the pain experience in HS. We speculate that low income may be associated with other social determinants of health for the patients assessed in this study, such as lack of social and community support or limited health care access that contribute to worse health outcomes.^10,11 In addition, low income contributes to limited access to medical care or treatments¹²; without access to effective HS management, lower-income patients may be at risk for higher disease severity and thus higher pain levels. However, economic stability is only a part of the whole picture; therefore, assessing the other social determinants of health in patients with HS may lead to better health outcomes and quality of life.

Another identified risk factor for high pain was a reported history of prescription pain medication use. This finding suggests that patients with moderate to severe pain likely have required stronger analgesic medications in the past. We further speculate that high pain levels in patients who have received prescription pain medications indicate either undertreatment, mistreatment, or recalcitrant pain. More research is needed to assess the relationship between HS-related pain intensity, analgesic medications, and providers who manage HS-related pain.

We also found that Black/African American patients with HS had a significantly higher dissatisfaction with their physician’s management of their pain, which could be attributable to several factors, including biological differences in medication metabolism (in which the patient has medication-resistant HS), undertreatment of pain, and/or poor doctor-patient relations. These reasons coincide with other diseases where health disparities are found.^13-15 Recognizing these factors will be key to dismantling the disparities in HS that are noted within this study. The limitations of this work include the cross-sectional study design and its inability to evaluate causal factors of high pain levels across racial groups, the NRS lack of insight on pain chronicity or pain experience,⁷ the lack of provider or institution perspectives, and self-reported data. Additionally, only patients with email access were included, which may have excluded vulnerable populations with more pain associated with their HS.

Our findings highlight an area for further investigation to assess why these racial differences exist in HS-related pain. The results also emphasize the need for research evaluating whether systemic or health care provider biases contribute to racial differences in HS-related pain management.

Acknowledgment — Dr. Weir was supported by the Predoctoral Clinical/Translational Research Program (TL1), a National Institutes of Health Ruth L. Kirschstein National Research Service Award (NRSA), through the University of Alabama at Birmingham (UAB) Center for Clinical and Translational Science (CCTS).  

To the Editor:

Psoriasis is an immune-mediated dermatologic condition that is associated with various comorbidities, including obesity.¹ The underlying pathophysiology of psoriasis has been extensively studied, and recent research has discussed the role of obesity in IL-17 secretion.² The relationship between being overweight/obese and having psoriasis has been documented in the literature.^1,2 However, this association in a recent population is lacking. We sought to investigate the association between psoriasis and obesity utilizing a representative US population of adults—the 2009-2014 National Health and Nutrition Examination Survey (NHANES) data,³ which contains the most recent psoriasis data.

We conducted a population-based, cross-sectional study focused on patients 20 years and older with psoriasis from the 2009-2014 NHANES database. Three 2-year cycles of NHANES data were combined to create our 2009 to 2014 dataset. In the Table, numerous variables including age, sex, household income, race/ethnicity, education, diabetes status, tobacco use, body mass index (BMI), waist circumference, and being called overweight by a health care provider were analyzed using χ² or t test analyses to evaluate for differences among those with and without psoriasis. Diabetes status was assessed by the question “Other than during pregnancy, have you ever been told by a doctor or health professional that you have diabetes or sugar diabetes?” Tobacco use was assessed by the question “Have you smoked at least 100 cigarettes in your entire life?” Psoriasis status was assessed by a self-reported response to the question “Have you ever been told by a doctor or other health care professional that you had psoriasis?” Three different outcome variables were used to determine if patients were overweight or obese: BMI, waist circumference, and response to the question “Has a doctor or other health professional ever told you that you were overweight?” Obesity was defined as having a BMI of 30 or higher or waist circumference of 102 cm or more in males and 88 cm or more in females.⁴ Being overweight was defined as having a BMI of 25 to 29.99 or response of Yes to “Has a doctor or other health professional ever told you that you were overweight?”

Initially, there were 17,547 participants 20 years and older from 2009 to 2014, but 1654 participants were excluded because of missing data for obesity or psoriasis; therefore, 15,893 patients were included in our analysis. Multivariable logistic regressions were utilized to examine the association between psoriasis and being overweight/obese (eTable). Additionally, the models were adjusted based on age, sex, household income, race/ethnicity, diabetes status, and tobacco use. All data processing and analysis were performed in Stata/MP 17 (StataCorp LLC). P<.05 was considered statistically significant.

The Table shows characteristics of US adults with and without psoriasis in NHANES 2009-2014. We found that the variables of interest evaluating body weight that were significantly different on analysis between patients with and without psoriasis included waist circumference—patients with psoriasis had a significantly higher waist circumference (P=.009)—and being told by a health care provider that they are overweight (P<.0001), which supports the findings by Love et al,⁵ who reported abdominal obesity was the most common feature of metabolic syndrome exhibited among patients with psoriasis.

Multivariable logistic regression analysis (eTable) revealed that there was a significant association between psoriasis and BMI of 25 to 29.99 (adjusted odds ratio [AOR], 1.34; 95% CI, 1.02-1.76; P=.04) and being told by a health care provider that they are overweight (AOR, 1.91; 95% CI, 1.44-2.52; P<.001). After adjusting for confounding variables, there was no significant association between psoriasis and a BMI of 30 or higher (AOR, 1.00; 95% CI, 0.73-1.38; P=.99) or a waist circumference of 102 cm or more in males and 88 cm or more in females (AOR, 1.15; 95% CI, 0.86-1.53; P=.3).

Our findings suggest that a few variables indicative of being overweight or obese are associated with psoriasis. This relationship most likely is due to increased adipokine, including resistin, levels in overweight individuals, resulting in a proinflammatory state.⁶ It has been suggested that BMI alone is not a definitive marker for measuring fat storage levels in individuals. People can have a normal or slightly elevated BMI but possess excessive adiposity, resulting in chronic inflammation.⁶ Therefore, our findings of a significant association between psoriasis and being told by a health care provider that they are overweight might be a stronger measurement for possessing excessive fat, as this is likely due to clinical judgment rather than BMI measurement.

Moreover, it should be noted that the potential reason for the lack of association between BMI of 30 or higher and psoriasis in our analysis may be a result of BMI serving as a poor measurement for adiposity. Additionally, Armstrong and colleagues⁷ discussed that the association between BMI and psoriasis was stronger for patients with moderate to severe psoriasis. Our study consisted of NHANES data for self-reported psoriasis diagnoses without a psoriasis severity index, making it difficult to extrapolate which individuals had mild or moderate to severe psoriasis, which may have contributed to our finding of no association between BMI of 30 or higher and psoriasis.

The self-reported nature of the survey questions and lack of questions regarding psoriasis severity serve as limitations to the study. Both obesity and psoriasis can have various systemic consequences, such as cardiovascular disease, due to the development of an inflammatory state.⁸ Future studies may explore other body measurements that indicate being overweight or obese and the potential synergistic relationship of obesity and psoriasis severity, optimizing the development of effective treatment plans.

To the Editor:

Psoriasis is an immune-mediated dermatologic condition that is associated with various comorbidities, including obesity.¹ The underlying pathophysiology of psoriasis has been extensively studied, and recent research has discussed the role of obesity in IL-17 secretion.² The relationship between being overweight/obese and having psoriasis has been documented in the literature.^1,2 However, this association in a recent population is lacking. We sought to investigate the association between psoriasis and obesity utilizing a representative US population of adults—the 2009-2014 National Health and Nutrition Examination Survey (NHANES) data,³ which contains the most recent psoriasis data.

We conducted a population-based, cross-sectional study focused on patients 20 years and older with psoriasis from the 2009-2014 NHANES database. Three 2-year cycles of NHANES data were combined to create our 2009 to 2014 dataset. In the Table, numerous variables including age, sex, household income, race/ethnicity, education, diabetes status, tobacco use, body mass index (BMI), waist circumference, and being called overweight by a health care provider were analyzed using χ² or t test analyses to evaluate for differences among those with and without psoriasis. Diabetes status was assessed by the question “Other than during pregnancy, have you ever been told by a doctor or health professional that you have diabetes or sugar diabetes?” Tobacco use was assessed by the question “Have you smoked at least 100 cigarettes in your entire life?” Psoriasis status was assessed by a self-reported response to the question “Have you ever been told by a doctor or other health care professional that you had psoriasis?” Three different outcome variables were used to determine if patients were overweight or obese: BMI, waist circumference, and response to the question “Has a doctor or other health professional ever told you that you were overweight?” Obesity was defined as having a BMI of 30 or higher or waist circumference of 102 cm or more in males and 88 cm or more in females.⁴ Being overweight was defined as having a BMI of 25 to 29.99 or response of Yes to “Has a doctor or other health professional ever told you that you were overweight?”

Initially, there were 17,547 participants 20 years and older from 2009 to 2014, but 1654 participants were excluded because of missing data for obesity or psoriasis; therefore, 15,893 patients were included in our analysis. Multivariable logistic regressions were utilized to examine the association between psoriasis and being overweight/obese (eTable). Additionally, the models were adjusted based on age, sex, household income, race/ethnicity, diabetes status, and tobacco use. All data processing and analysis were performed in Stata/MP 17 (StataCorp LLC). P<.05 was considered statistically significant.

The Table shows characteristics of US adults with and without psoriasis in NHANES 2009-2014. We found that the variables of interest evaluating body weight that were significantly different on analysis between patients with and without psoriasis included waist circumference—patients with psoriasis had a significantly higher waist circumference (P=.009)—and being told by a health care provider that they are overweight (P<.0001), which supports the findings by Love et al,⁵ who reported abdominal obesity was the most common feature of metabolic syndrome exhibited among patients with psoriasis.

Multivariable logistic regression analysis (eTable) revealed that there was a significant association between psoriasis and BMI of 25 to 29.99 (adjusted odds ratio [AOR], 1.34; 95% CI, 1.02-1.76; P=.04) and being told by a health care provider that they are overweight (AOR, 1.91; 95% CI, 1.44-2.52; P<.001). After adjusting for confounding variables, there was no significant association between psoriasis and a BMI of 30 or higher (AOR, 1.00; 95% CI, 0.73-1.38; P=.99) or a waist circumference of 102 cm or more in males and 88 cm or more in females (AOR, 1.15; 95% CI, 0.86-1.53; P=.3).

Our findings suggest that a few variables indicative of being overweight or obese are associated with psoriasis. This relationship most likely is due to increased adipokine, including resistin, levels in overweight individuals, resulting in a proinflammatory state.⁶ It has been suggested that BMI alone is not a definitive marker for measuring fat storage levels in individuals. People can have a normal or slightly elevated BMI but possess excessive adiposity, resulting in chronic inflammation.⁶ Therefore, our findings of a significant association between psoriasis and being told by a health care provider that they are overweight might be a stronger measurement for possessing excessive fat, as this is likely due to clinical judgment rather than BMI measurement.

Moreover, it should be noted that the potential reason for the lack of association between BMI of 30 or higher and psoriasis in our analysis may be a result of BMI serving as a poor measurement for adiposity. Additionally, Armstrong and colleagues⁷ discussed that the association between BMI and psoriasis was stronger for patients with moderate to severe psoriasis. Our study consisted of NHANES data for self-reported psoriasis diagnoses without a psoriasis severity index, making it difficult to extrapolate which individuals had mild or moderate to severe psoriasis, which may have contributed to our finding of no association between BMI of 30 or higher and psoriasis.

The self-reported nature of the survey questions and lack of questions regarding psoriasis severity serve as limitations to the study. Both obesity and psoriasis can have various systemic consequences, such as cardiovascular disease, due to the development of an inflammatory state.⁸ Future studies may explore other body measurements that indicate being overweight or obese and the potential synergistic relationship of obesity and psoriasis severity, optimizing the development of effective treatment plans.

To the Editor:

Psoriasis is an immune-mediated dermatologic condition that is associated with various comorbidities, including obesity.¹ The underlying pathophysiology of psoriasis has been extensively studied, and recent research has discussed the role of obesity in IL-17 secretion.² The relationship between being overweight/obese and having psoriasis has been documented in the literature.^1,2 However, this association in a recent population is lacking. We sought to investigate the association between psoriasis and obesity utilizing a representative US population of adults—the 2009-2014 National Health and Nutrition Examination Survey (NHANES) data,³ which contains the most recent psoriasis data.

We conducted a population-based, cross-sectional study focused on patients 20 years and older with psoriasis from the 2009-2014 NHANES database. Three 2-year cycles of NHANES data were combined to create our 2009 to 2014 dataset. In the Table, numerous variables including age, sex, household income, race/ethnicity, education, diabetes status, tobacco use, body mass index (BMI), waist circumference, and being called overweight by a health care provider were analyzed using χ² or t test analyses to evaluate for differences among those with and without psoriasis. Diabetes status was assessed by the question “Other than during pregnancy, have you ever been told by a doctor or health professional that you have diabetes or sugar diabetes?” Tobacco use was assessed by the question “Have you smoked at least 100 cigarettes in your entire life?” Psoriasis status was assessed by a self-reported response to the question “Have you ever been told by a doctor or other health care professional that you had psoriasis?” Three different outcome variables were used to determine if patients were overweight or obese: BMI, waist circumference, and response to the question “Has a doctor or other health professional ever told you that you were overweight?” Obesity was defined as having a BMI of 30 or higher or waist circumference of 102 cm or more in males and 88 cm or more in females.⁴ Being overweight was defined as having a BMI of 25 to 29.99 or response of Yes to “Has a doctor or other health professional ever told you that you were overweight?”

Initially, there were 17,547 participants 20 years and older from 2009 to 2014, but 1654 participants were excluded because of missing data for obesity or psoriasis; therefore, 15,893 patients were included in our analysis. Multivariable logistic regressions were utilized to examine the association between psoriasis and being overweight/obese (eTable). Additionally, the models were adjusted based on age, sex, household income, race/ethnicity, diabetes status, and tobacco use. All data processing and analysis were performed in Stata/MP 17 (StataCorp LLC). P<.05 was considered statistically significant.

The Table shows characteristics of US adults with and without psoriasis in NHANES 2009-2014. We found that the variables of interest evaluating body weight that were significantly different on analysis between patients with and without psoriasis included waist circumference—patients with psoriasis had a significantly higher waist circumference (P=.009)—and being told by a health care provider that they are overweight (P<.0001), which supports the findings by Love et al,⁵ who reported abdominal obesity was the most common feature of metabolic syndrome exhibited among patients with psoriasis.

Multivariable logistic regression analysis (eTable) revealed that there was a significant association between psoriasis and BMI of 25 to 29.99 (adjusted odds ratio [AOR], 1.34; 95% CI, 1.02-1.76; P=.04) and being told by a health care provider that they are overweight (AOR, 1.91; 95% CI, 1.44-2.52; P<.001). After adjusting for confounding variables, there was no significant association between psoriasis and a BMI of 30 or higher (AOR, 1.00; 95% CI, 0.73-1.38; P=.99) or a waist circumference of 102 cm or more in males and 88 cm or more in females (AOR, 1.15; 95% CI, 0.86-1.53; P=.3).

Our findings suggest that a few variables indicative of being overweight or obese are associated with psoriasis. This relationship most likely is due to increased adipokine, including resistin, levels in overweight individuals, resulting in a proinflammatory state.⁶ It has been suggested that BMI alone is not a definitive marker for measuring fat storage levels in individuals. People can have a normal or slightly elevated BMI but possess excessive adiposity, resulting in chronic inflammation.⁶ Therefore, our findings of a significant association between psoriasis and being told by a health care provider that they are overweight might be a stronger measurement for possessing excessive fat, as this is likely due to clinical judgment rather than BMI measurement.

Moreover, it should be noted that the potential reason for the lack of association between BMI of 30 or higher and psoriasis in our analysis may be a result of BMI serving as a poor measurement for adiposity. Additionally, Armstrong and colleagues⁷ discussed that the association between BMI and psoriasis was stronger for patients with moderate to severe psoriasis. Our study consisted of NHANES data for self-reported psoriasis diagnoses without a psoriasis severity index, making it difficult to extrapolate which individuals had mild or moderate to severe psoriasis, which may have contributed to our finding of no association between BMI of 30 or higher and psoriasis.

The self-reported nature of the survey questions and lack of questions regarding psoriasis severity serve as limitations to the study. Both obesity and psoriasis can have various systemic consequences, such as cardiovascular disease, due to the development of an inflammatory state.⁸ Future studies may explore other body measurements that indicate being overweight or obese and the potential synergistic relationship of obesity and psoriasis severity, optimizing the development of effective treatment plans.

Palliative care (PC) is a field of medicine that focuses on improving quality of life by managing physical symptoms as well as mental and spiritual well-being in patients with severe illnesses.^1,2 Despite cases of severe dermatologic disease, the use of PC in the field of dermatology is limited, often leaving patients with a range of unmet needs.^2,3 In one study that explored PC in patients with melanoma, only one-third of patients with advanced melanoma had a PC consultation.⁴ Reasons behind the lack of utilization of PC in dermatology include time constraints and limited training in addressing the complex psychosocial needs of patients with severe dermatologic illnesses.¹ We conducted a retrospective, cross-sectional, single-institution study of specific inpatient dermatology consultations over a 5-year period to describe PC utilization among patients who were hospitalized with select severe dermatologic diseases.

Methods

A retrospective, cross-sectional study of inpatient dermatology consultations over a 5-year period (October 2016 to October 2021) was performed at Atrium Health Wake Forest Baptist Medical Center (Winston-Salem, North Carolina). Patients’ medical records were reviewed if they had one of the following diseases: bullous pemphigoid, calciphylaxis, cutaneous T-cell lymphoma (CTCL), drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome, erythrodermic psoriasis, graft-vs-host disease, pemphigus vulgaris (PV), purpura fulminans, pyoderma gangrenosum, and Stevens-Johnson syndrome/toxic epidermal necrolysis. These diseases were selected for inclusion because they have been associated with a documented increase in inpatient mortality and have been described in the published literature on PC in dermatology.² This study was reviewed and approved by the Wake Forest University institutional review board.

Use of PC consultative services along with other associated consultative care (ie, recreation therapy [RT], acute pain management, pastoral care) was assessed for each patient. Recreation therapy included specific interventions such as music therapy, arts/craft therapy, pet therapy, and other services with the goal of improving patient cognitive, emotional, and social function. For patients with a completed PC consultation, goals for PC intervention were recorded.

Results

The total study sample included 193 inpatient dermatology consultations. The mean age of the patients was 58.9 years (range, 2–100 years); 66.8% (129/193) were White and 28.5% (55/193) were Black (Table). Palliative care was consulted in 5.7% of cases, with consultations being requested by the primary care team. Reasons for PC consultation included assessment of the patient’s goals of care (4.1% [8/193]), pain management (3.6% [7/193]), non–pain symptom management (2.6% [5/193]), psychosocial support (1.6% [3/193]), and transitions of care (1.0% [2/193]). The average length of patients’ hospital stay prior to PC consultation was 11.5 days(range, 1–32 days). Acute pain management was the reason for consultation in 15.0% of cases (29/193), RT in 21.8% (42/193), and pastoral care in 13.5% (26/193) of cases. Patients with calciphylaxis received the most PC and pain consultations, but fewer than half received these services. Patients with calciphylaxis, PV, purpura fulminans, and CTCL received a higher percentage of PC consultations than the overall cohort, while patients with calciphylaxis, DRESS syndrome, PV, and pyoderma gangrenosum received relatively more pain consultations than the overall cohort (Figure).

Comment

Clinical practice guidelines for quality PC stress the importance of specialists being familiar with these services and the ability to involve PC as part of the treatment plan to achieve better care for patients with serious illnesses.⁵ Our results demonstrated low rates of PC consultation services for dermatology patients, which supports the existing literature and suggests that PC may be highly underutilized in inpatient settings for patients with serious skin diseases. Use of PC was infrequent and was initiated relatively late in the course of hospital admission, which can negatively impact a patient’s well-being and care experience and can increase the care burden on their caregivers and families.²

Our results suggest a discrepancy in the frequency of formal PC and other palliative consultative services used for dermatologic diseases, with non-PC services including RT, acute pain management, and pastoral care more likely to be utilized. Impacting this finding may be that RT, pastoral care, and acute pain management are provided by nonphysician providers at our institution, not attending faculty staffing PC services. Patients with calciphylaxis were more likely to have PC consultations, potentially due to medicine providers’ familiarity with its morbidity and mortality, as it is commonly associated with end-stage renal disease. Similarly, internal medicine providers may be more familiar with pain classically associated with PG and PV and may be more likely to engage pain experts. Some diseases with notable morbidity and potential mortality were underrepresented including SJS/TEN, erythrodermic psoriasis, CTCL, and GVHD.

Limitations of our study included examination of data from a single institution, as well as the small sample sizes in specific subgroups, which prevented us from making comparisons between diseases. The cross-sectional design also limited our ability to control for confounding variables.

Conclusion

We urge dermatology consultation services to advocate for patients with serious skin diseases andinclude PC consultation as part of their recommendations to primary care teams. Further research should characterize the specific needs of patients that may be addressed by PC services and explore ways dermatologists and others can identify and provide specialty care to hospitalized patients.

Palliative care (PC) is a field of medicine that focuses on improving quality of life by managing physical symptoms as well as mental and spiritual well-being in patients with severe illnesses.^1,2 Despite cases of severe dermatologic disease, the use of PC in the field of dermatology is limited, often leaving patients with a range of unmet needs.^2,3 In one study that explored PC in patients with melanoma, only one-third of patients with advanced melanoma had a PC consultation.⁴ Reasons behind the lack of utilization of PC in dermatology include time constraints and limited training in addressing the complex psychosocial needs of patients with severe dermatologic illnesses.¹ We conducted a retrospective, cross-sectional, single-institution study of specific inpatient dermatology consultations over a 5-year period to describe PC utilization among patients who were hospitalized with select severe dermatologic diseases.

Methods

A retrospective, cross-sectional study of inpatient dermatology consultations over a 5-year period (October 2016 to October 2021) was performed at Atrium Health Wake Forest Baptist Medical Center (Winston-Salem, North Carolina). Patients’ medical records were reviewed if they had one of the following diseases: bullous pemphigoid, calciphylaxis, cutaneous T-cell lymphoma (CTCL), drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome, erythrodermic psoriasis, graft-vs-host disease, pemphigus vulgaris (PV), purpura fulminans, pyoderma gangrenosum, and Stevens-Johnson syndrome/toxic epidermal necrolysis. These diseases were selected for inclusion because they have been associated with a documented increase in inpatient mortality and have been described in the published literature on PC in dermatology.² This study was reviewed and approved by the Wake Forest University institutional review board.

Use of PC consultative services along with other associated consultative care (ie, recreation therapy [RT], acute pain management, pastoral care) was assessed for each patient. Recreation therapy included specific interventions such as music therapy, arts/craft therapy, pet therapy, and other services with the goal of improving patient cognitive, emotional, and social function. For patients with a completed PC consultation, goals for PC intervention were recorded.

Results

The total study sample included 193 inpatient dermatology consultations. The mean age of the patients was 58.9 years (range, 2–100 years); 66.8% (129/193) were White and 28.5% (55/193) were Black (Table). Palliative care was consulted in 5.7% of cases, with consultations being requested by the primary care team. Reasons for PC consultation included assessment of the patient’s goals of care (4.1% [8/193]), pain management (3.6% [7/193]), non–pain symptom management (2.6% [5/193]), psychosocial support (1.6% [3/193]), and transitions of care (1.0% [2/193]). The average length of patients’ hospital stay prior to PC consultation was 11.5 days(range, 1–32 days). Acute pain management was the reason for consultation in 15.0% of cases (29/193), RT in 21.8% (42/193), and pastoral care in 13.5% (26/193) of cases. Patients with calciphylaxis received the most PC and pain consultations, but fewer than half received these services. Patients with calciphylaxis, PV, purpura fulminans, and CTCL received a higher percentage of PC consultations than the overall cohort, while patients with calciphylaxis, DRESS syndrome, PV, and pyoderma gangrenosum received relatively more pain consultations than the overall cohort (Figure).

Comment

Clinical practice guidelines for quality PC stress the importance of specialists being familiar with these services and the ability to involve PC as part of the treatment plan to achieve better care for patients with serious illnesses.⁵ Our results demonstrated low rates of PC consultation services for dermatology patients, which supports the existing literature and suggests that PC may be highly underutilized in inpatient settings for patients with serious skin diseases. Use of PC was infrequent and was initiated relatively late in the course of hospital admission, which can negatively impact a patient’s well-being and care experience and can increase the care burden on their caregivers and families.²

Our results suggest a discrepancy in the frequency of formal PC and other palliative consultative services used for dermatologic diseases, with non-PC services including RT, acute pain management, and pastoral care more likely to be utilized. Impacting this finding may be that RT, pastoral care, and acute pain management are provided by nonphysician providers at our institution, not attending faculty staffing PC services. Patients with calciphylaxis were more likely to have PC consultations, potentially due to medicine providers’ familiarity with its morbidity and mortality, as it is commonly associated with end-stage renal disease. Similarly, internal medicine providers may be more familiar with pain classically associated with PG and PV and may be more likely to engage pain experts. Some diseases with notable morbidity and potential mortality were underrepresented including SJS/TEN, erythrodermic psoriasis, CTCL, and GVHD.

Limitations of our study included examination of data from a single institution, as well as the small sample sizes in specific subgroups, which prevented us from making comparisons between diseases. The cross-sectional design also limited our ability to control for confounding variables.

Conclusion

We urge dermatology consultation services to advocate for patients with serious skin diseases andinclude PC consultation as part of their recommendations to primary care teams. Further research should characterize the specific needs of patients that may be addressed by PC services and explore ways dermatologists and others can identify and provide specialty care to hospitalized patients.

Palliative care (PC) is a field of medicine that focuses on improving quality of life by managing physical symptoms as well as mental and spiritual well-being in patients with severe illnesses.^1,2 Despite cases of severe dermatologic disease, the use of PC in the field of dermatology is limited, often leaving patients with a range of unmet needs.^2,3 In one study that explored PC in patients with melanoma, only one-third of patients with advanced melanoma had a PC consultation.⁴ Reasons behind the lack of utilization of PC in dermatology include time constraints and limited training in addressing the complex psychosocial needs of patients with severe dermatologic illnesses.¹ We conducted a retrospective, cross-sectional, single-institution study of specific inpatient dermatology consultations over a 5-year period to describe PC utilization among patients who were hospitalized with select severe dermatologic diseases.

Methods

A retrospective, cross-sectional study of inpatient dermatology consultations over a 5-year period (October 2016 to October 2021) was performed at Atrium Health Wake Forest Baptist Medical Center (Winston-Salem, North Carolina). Patients’ medical records were reviewed if they had one of the following diseases: bullous pemphigoid, calciphylaxis, cutaneous T-cell lymphoma (CTCL), drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome, erythrodermic psoriasis, graft-vs-host disease, pemphigus vulgaris (PV), purpura fulminans, pyoderma gangrenosum, and Stevens-Johnson syndrome/toxic epidermal necrolysis. These diseases were selected for inclusion because they have been associated with a documented increase in inpatient mortality and have been described in the published literature on PC in dermatology.² This study was reviewed and approved by the Wake Forest University institutional review board.

Use of PC consultative services along with other associated consultative care (ie, recreation therapy [RT], acute pain management, pastoral care) was assessed for each patient. Recreation therapy included specific interventions such as music therapy, arts/craft therapy, pet therapy, and other services with the goal of improving patient cognitive, emotional, and social function. For patients with a completed PC consultation, goals for PC intervention were recorded.

Results

The total study sample included 193 inpatient dermatology consultations. The mean age of the patients was 58.9 years (range, 2–100 years); 66.8% (129/193) were White and 28.5% (55/193) were Black (Table). Palliative care was consulted in 5.7% of cases, with consultations being requested by the primary care team. Reasons for PC consultation included assessment of the patient’s goals of care (4.1% [8/193]), pain management (3.6% [7/193]), non–pain symptom management (2.6% [5/193]), psychosocial support (1.6% [3/193]), and transitions of care (1.0% [2/193]). The average length of patients’ hospital stay prior to PC consultation was 11.5 days(range, 1–32 days). Acute pain management was the reason for consultation in 15.0% of cases (29/193), RT in 21.8% (42/193), and pastoral care in 13.5% (26/193) of cases. Patients with calciphylaxis received the most PC and pain consultations, but fewer than half received these services. Patients with calciphylaxis, PV, purpura fulminans, and CTCL received a higher percentage of PC consultations than the overall cohort, while patients with calciphylaxis, DRESS syndrome, PV, and pyoderma gangrenosum received relatively more pain consultations than the overall cohort (Figure).

Comment

Clinical practice guidelines for quality PC stress the importance of specialists being familiar with these services and the ability to involve PC as part of the treatment plan to achieve better care for patients with serious illnesses.⁵ Our results demonstrated low rates of PC consultation services for dermatology patients, which supports the existing literature and suggests that PC may be highly underutilized in inpatient settings for patients with serious skin diseases. Use of PC was infrequent and was initiated relatively late in the course of hospital admission, which can negatively impact a patient’s well-being and care experience and can increase the care burden on their caregivers and families.²

Our results suggest a discrepancy in the frequency of formal PC and other palliative consultative services used for dermatologic diseases, with non-PC services including RT, acute pain management, and pastoral care more likely to be utilized. Impacting this finding may be that RT, pastoral care, and acute pain management are provided by nonphysician providers at our institution, not attending faculty staffing PC services. Patients with calciphylaxis were more likely to have PC consultations, potentially due to medicine providers’ familiarity with its morbidity and mortality, as it is commonly associated with end-stage renal disease. Similarly, internal medicine providers may be more familiar with pain classically associated with PG and PV and may be more likely to engage pain experts. Some diseases with notable morbidity and potential mortality were underrepresented including SJS/TEN, erythrodermic psoriasis, CTCL, and GVHD.

Limitations of our study included examination of data from a single institution, as well as the small sample sizes in specific subgroups, which prevented us from making comparisons between diseases. The cross-sectional design also limited our ability to control for confounding variables.

Conclusion

We urge dermatology consultation services to advocate for patients with serious skin diseases andinclude PC consultation as part of their recommendations to primary care teams. Further research should characterize the specific needs of patients that may be addressed by PC services and explore ways dermatologists and others can identify and provide specialty care to hospitalized patients.

The Diagnosis: Diffuse Dermal Angiomatosis

Diffuse dermal angiomatosis (DDA) is an acquired reactive vascular proliferation in the spectrum of cutaneous reactive angioendotheliomatoses. Clinically, DDA presents as violaceous reticulated plaques, often with secondary ulceration and sometimes necrosis.1-3 Diffuse dermal angiomatosis more commonly presents in patients with a history of severe peripheral vascular disease, coagulopathies, or infection, and it frequently arises on the extremities. Diffuse dermal angiomatosis also has been shown to develop on the breasts, particularly in patients with pendulous breast tissue. Vascular proliferation in DDA is hypothesized to be from ischemia and hypoxia, leading to angiogenesis.^1-3 Diffuse dermal angiomatosis is characterized histologically by the presence of a diffuse proliferation of spindled endothelial cells distributed between the collagen bundles throughout the dermis (quiz image and Figure 1). Spindle-shaped endothelial cells exhibit a vacuolated cytoplasm. On immunohistochemistry, these dermal spindle cells classically stain positive for CD31, CD34, and erythroblast transformation specific–related gene (Erg) and stain negative for both human herpesvirus 8 (HHV-8) and factor XIIIa.

Cutaneous fibrous histiocytoma, more commonly referred to as dermatofibroma, is a common benign lesion that presents clinically as a solitary firm nodule most commonly on the extremities in areas of repetitive trauma or pressure. It classically exhibits dimpling of the overlaying skin with lateral pressure on the lesion, known as the dimple sign.⁴ Histologically, dermatofibromas share similar features to DDA and demonstrate the presence of bland-appearing spindle cells within the dermis between the collagen bundles, resulting in collagen trapping. However, a distinguishing histologic feature of a dermatofibroma in comparison to DDA is the presence of epidermal hyperplasia overlying the dermatofibroma, leading to tabled rete ridges (Figure 2). Spindle cells in dermatofibromas are fibroblasts and have a distinct immunophenotype that includes factor XIIIa positivity and negative staining for CD31, CD34, and Erg.^4,5

Dermatofibrosarcoma protuberans (DFSP) is a rare malignant soft-tissue sarcoma that clinically presents as a firm, flesh-colored, dermal plaque on the trunk, proximal extremities, head, or neck.⁵ Histologically, DFSP can be distinguished from DDA by the high density of spindle cells that are arranged in a storiform pattern, extending and infiltrating the underlying subcutaneous fat in a honeycomblike pattern (Figure 3). Spindle cells in DFSP typically show expression of CD34 but are negative for CD31, Erg, and factor XIIIa.⁵

Kaposi sarcoma (KS) is an endothelial cell–driven angioproliferative neoplasm that is associated with HHV-8 infection.⁶ The clinical presentation of KS can range from isolated pink or purple papules and patches to more extensive ulcerated plaques or nodules. Histopathology exhibits proliferation of monomorphic spindled endothelial cells within the dermis staining positive for HHV-8, Erg, CD31, and CD34, in conjunction with extravasated erythrocytes arranged within slitlike vascular spaces (Figure 4). Additionally, KS classically exhibits aberrant endothelial cell proliferation and vessel formation around preexisting vessels, which is referred to as the promontory sign (Figure 4).

Angiosarcoma is a rare and highly aggressive vascular tumor arising from endothelial cells lining the blood vessels and lymphatics.^7,8 Clinically, angiosarcoma presents as ulcerated violaceous nodules or plaques on the head, neck, or trunk. Histologic evaluation of angiosarcoma reveals a complex and poorly demarcated vascular network dissecting between collagen bundles in the dermis (Figure 5). Multilayering of endothelial cells, papillary projections extending into the vessel lumina, and mitoses frequently are seen. On immunohistochemistry, endothelial cells demonstrate prominent cellular atypia and stain positive with CD31, CD34, and Erg.

The Diagnosis: Diffuse Dermal Angiomatosis

Diffuse dermal angiomatosis (DDA) is an acquired reactive vascular proliferation in the spectrum of cutaneous reactive angioendotheliomatoses. Clinically, DDA presents as violaceous reticulated plaques, often with secondary ulceration and sometimes necrosis.1-3 Diffuse dermal angiomatosis more commonly presents in patients with a history of severe peripheral vascular disease, coagulopathies, or infection, and it frequently arises on the extremities. Diffuse dermal angiomatosis also has been shown to develop on the breasts, particularly in patients with pendulous breast tissue. Vascular proliferation in DDA is hypothesized to be from ischemia and hypoxia, leading to angiogenesis.^1-3 Diffuse dermal angiomatosis is characterized histologically by the presence of a diffuse proliferation of spindled endothelial cells distributed between the collagen bundles throughout the dermis (quiz image and Figure 1). Spindle-shaped endothelial cells exhibit a vacuolated cytoplasm. On immunohistochemistry, these dermal spindle cells classically stain positive for CD31, CD34, and erythroblast transformation specific–related gene (Erg) and stain negative for both human herpesvirus 8 (HHV-8) and factor XIIIa.

Cutaneous fibrous histiocytoma, more commonly referred to as dermatofibroma, is a common benign lesion that presents clinically as a solitary firm nodule most commonly on the extremities in areas of repetitive trauma or pressure. It classically exhibits dimpling of the overlaying skin with lateral pressure on the lesion, known as the dimple sign.⁴ Histologically, dermatofibromas share similar features to DDA and demonstrate the presence of bland-appearing spindle cells within the dermis between the collagen bundles, resulting in collagen trapping. However, a distinguishing histologic feature of a dermatofibroma in comparison to DDA is the presence of epidermal hyperplasia overlying the dermatofibroma, leading to tabled rete ridges (Figure 2). Spindle cells in dermatofibromas are fibroblasts and have a distinct immunophenotype that includes factor XIIIa positivity and negative staining for CD31, CD34, and Erg.^4,5

Dermatofibrosarcoma protuberans (DFSP) is a rare malignant soft-tissue sarcoma that clinically presents as a firm, flesh-colored, dermal plaque on the trunk, proximal extremities, head, or neck.⁵ Histologically, DFSP can be distinguished from DDA by the high density of spindle cells that are arranged in a storiform pattern, extending and infiltrating the underlying subcutaneous fat in a honeycomblike pattern (Figure 3). Spindle cells in DFSP typically show expression of CD34 but are negative for CD31, Erg, and factor XIIIa.⁵

Kaposi sarcoma (KS) is an endothelial cell–driven angioproliferative neoplasm that is associated with HHV-8 infection.⁶ The clinical presentation of KS can range from isolated pink or purple papules and patches to more extensive ulcerated plaques or nodules. Histopathology exhibits proliferation of monomorphic spindled endothelial cells within the dermis staining positive for HHV-8, Erg, CD31, and CD34, in conjunction with extravasated erythrocytes arranged within slitlike vascular spaces (Figure 4). Additionally, KS classically exhibits aberrant endothelial cell proliferation and vessel formation around preexisting vessels, which is referred to as the promontory sign (Figure 4).

Angiosarcoma is a rare and highly aggressive vascular tumor arising from endothelial cells lining the blood vessels and lymphatics.^7,8 Clinically, angiosarcoma presents as ulcerated violaceous nodules or plaques on the head, neck, or trunk. Histologic evaluation of angiosarcoma reveals a complex and poorly demarcated vascular network dissecting between collagen bundles in the dermis (Figure 5). Multilayering of endothelial cells, papillary projections extending into the vessel lumina, and mitoses frequently are seen. On immunohistochemistry, endothelial cells demonstrate prominent cellular atypia and stain positive with CD31, CD34, and Erg.

The Diagnosis: Diffuse Dermal Angiomatosis

Diffuse dermal angiomatosis (DDA) is an acquired reactive vascular proliferation in the spectrum of cutaneous reactive angioendotheliomatoses. Clinically, DDA presents as violaceous reticulated plaques, often with secondary ulceration and sometimes necrosis.1-3 Diffuse dermal angiomatosis more commonly presents in patients with a history of severe peripheral vascular disease, coagulopathies, or infection, and it frequently arises on the extremities. Diffuse dermal angiomatosis also has been shown to develop on the breasts, particularly in patients with pendulous breast tissue. Vascular proliferation in DDA is hypothesized to be from ischemia and hypoxia, leading to angiogenesis.^1-3 Diffuse dermal angiomatosis is characterized histologically by the presence of a diffuse proliferation of spindled endothelial cells distributed between the collagen bundles throughout the dermis (quiz image and Figure 1). Spindle-shaped endothelial cells exhibit a vacuolated cytoplasm. On immunohistochemistry, these dermal spindle cells classically stain positive for CD31, CD34, and erythroblast transformation specific–related gene (Erg) and stain negative for both human herpesvirus 8 (HHV-8) and factor XIIIa.

Cutaneous fibrous histiocytoma, more commonly referred to as dermatofibroma, is a common benign lesion that presents clinically as a solitary firm nodule most commonly on the extremities in areas of repetitive trauma or pressure. It classically exhibits dimpling of the overlaying skin with lateral pressure on the lesion, known as the dimple sign.⁴ Histologically, dermatofibromas share similar features to DDA and demonstrate the presence of bland-appearing spindle cells within the dermis between the collagen bundles, resulting in collagen trapping. However, a distinguishing histologic feature of a dermatofibroma in comparison to DDA is the presence of epidermal hyperplasia overlying the dermatofibroma, leading to tabled rete ridges (Figure 2). Spindle cells in dermatofibromas are fibroblasts and have a distinct immunophenotype that includes factor XIIIa positivity and negative staining for CD31, CD34, and Erg.^4,5

Dermatofibrosarcoma protuberans (DFSP) is a rare malignant soft-tissue sarcoma that clinically presents as a firm, flesh-colored, dermal plaque on the trunk, proximal extremities, head, or neck.⁵ Histologically, DFSP can be distinguished from DDA by the high density of spindle cells that are arranged in a storiform pattern, extending and infiltrating the underlying subcutaneous fat in a honeycomblike pattern (Figure 3). Spindle cells in DFSP typically show expression of CD34 but are negative for CD31, Erg, and factor XIIIa.⁵

Kaposi sarcoma (KS) is an endothelial cell–driven angioproliferative neoplasm that is associated with HHV-8 infection.⁶ The clinical presentation of KS can range from isolated pink or purple papules and patches to more extensive ulcerated plaques or nodules. Histopathology exhibits proliferation of monomorphic spindled endothelial cells within the dermis staining positive for HHV-8, Erg, CD31, and CD34, in conjunction with extravasated erythrocytes arranged within slitlike vascular spaces (Figure 4). Additionally, KS classically exhibits aberrant endothelial cell proliferation and vessel formation around preexisting vessels, which is referred to as the promontory sign (Figure 4).

Angiosarcoma is a rare and highly aggressive vascular tumor arising from endothelial cells lining the blood vessels and lymphatics.^7,8 Clinically, angiosarcoma presents as ulcerated violaceous nodules or plaques on the head, neck, or trunk. Histologic evaluation of angiosarcoma reveals a complex and poorly demarcated vascular network dissecting between collagen bundles in the dermis (Figure 5). Multilayering of endothelial cells, papillary projections extending into the vessel lumina, and mitoses frequently are seen. On immunohistochemistry, endothelial cells demonstrate prominent cellular atypia and stain positive with CD31, CD34, and Erg.

Dermatologic disparities disproportionately affect patients with skin of color (SOC). Two studies assessing the diagnostic accuracy of medical students have shown disparities in diagnosing common skin conditions presenting in darker skin compared to lighter skin at early stages of training.^1,2 This knowledge gap could be attributed to the underrepresentation of SOC in dermatologic textbooks, journals, and educational curricula.^3-6 It is important for dermatologists as well as physicians in other specialties and ancillary health care workers involved in treating or triaging dermatologic diseases to recognize common skin conditions presenting in SOC. We sought to evaluate the effectiveness of a focused educational module for improving diagnostic accuracy and confidence in treating SOC among interprofessional health care providers.

Methods

Interprofessional health care providers—medical students, residents/fellows, attending physicians, advanced practice providers (APPs), and nurses practicing across various medical specialties—at The University of Texas at Austin Dell Medical School and Ascension Medical Group (both in Austin, Texas) were invited to participate in an institutional review board–exempt study involving a virtual SOC educational module from February through May 2021. The 1-hour module involved a pretest, a 15-minute lecture, an immediate posttest, and a 3-month posttest. All tests included the same 40 multiple-choice questions of 20 dermatologic conditions portrayed in lighter and darker skin types from VisualDx.com, and participants were asked to identify the condition in each photograph. Questions appeared one at a time in a randomized order, and answers could not be changed once submitted.

For analysis, the dermatologic conditions were categorized into 4 groups: cancerous, infectious, inflammatory, and SOC-associated conditions. Cancerous conditions included basal cell carcinoma, squamous cell carcinoma, and melanoma. Infectious conditions included herpes zoster, tinea corporis, tinea versicolor, staphylococcal scalded skin syndrome, and verruca vulgaris. Inflammatory conditions included acne, atopic dermatitis, pityriasis rosea, psoriasis, seborrheic dermatitis, contact dermatitis, lichen planus, and urticaria. Skin of color–associated conditions included hidradenitis suppurativa, acanthosis nigricans, keloid, and melasma. Two questions utilizing a 5-point Likert scale assessing confidence in diagnosing light and dark skin also were included.

The pre-recorded 15-minute video lecture was given by 2 dermatology residents (P.L.K. and C.P.), and the learning objectives covered morphologic differences in lighter skin and darker skin, comparisons of common dermatologic diseases in lighter skin and darker skin, diseases more commonly affecting patients with SOC, and treatment considerations for conditions affecting skin and hair in patients with SOC. Photographs from the diagnostic accuracy assessment were not reused in the lecture. Detailed explanations on morphology, diagnostic pearls, and treatment options for all conditions tested were provided to participants upon completion of the 3-month posttest.

Statistical Analysis—Test scores were compared between conditions shown in lighter and darker skin types and from the pretest to the immediate posttest and 3-month posttest. Multiple linear regression was used to assess for intervention effects on lighter and darker skin scores controlling for provider type and specialty. All tests were 2-sided with significance at P<.05. Analyses were conducted using Stata 17.

Results

One hundred participants completed the pretest and immediate posttest, 36 of whom also completed the 3-month posttest (Table). There was no significant difference in baseline characteristics between the pretest and 3-month posttest groups.

Test scores were correlated with provider type and specialty but not age, sex, or race/ethnicity. Specializing in dermatology and being a resident or attending physician were independently associated with higher test scores. Mean pretest diagnostic accuracy and confidence scores were higher for skin conditions shown in lighter skin compared with those shown in darker skin (13.6 vs 11.3 and 2.7 vs 1.9, respectively; both P<.001). Pretest diagnostic accuracy was significantly higher for skin conditions shown in lighter skin compared with darker skin for cancerous, inflammatory, and infectious conditions (72% vs 50%, 68% vs 55%, and 57% vs 47%, respectively; P<.001 for all)(Figure 1). Skin of color–associated conditions were not associated with significantly different scores for lighter skin compared with darker skin (79% vs 75%; P=.059).

Controlling for provider type and specialty, significantly improved diagnostic accuracy was seen in immediate posttest scores compared with pretest scores for conditions shown in both lighter and darker skin types (lighter: 15.2 vs 13.6; darker: 13.3 vs 11.3; both P<.001)(Figure 2). The immediate posttest demonstrated higher mean diagnostic accuracy and confidence scores for skin conditions shown in lighter skin compared with darker skin (diagnostic accuracy: 15.2 vs 13.3; confidence: 3.0 vs 2.6; both P<.001), but the disparity between scores was less than in the pretest.

Following the 3-month posttest, improvement in diagnostic accuracy was noted among both lighter and darker skin types compared with the pretest, but the difference remained significant only for conditions shown in darker skin (mean scores, 11.3 vs 13.3; P<.01). Similarly, confidence in diagnosing conditions in both lighter and darker skin improved following the immediate posttest (mean scores, 2.7 vs 3.0 and 1.9 vs 2.6; both P<.001), and this improvement remained significant for only darker skin following the 3-month posttest (mean scores, 1.9 vs 2.3; P<.001). Despite these improvements, diagnostic accuracy and confidence remained higher for skin conditions shown in lighter skin compared with darker skin (diagnostic accuracy: 14.7 vs 13.3; P<.01; confidence: 2.8 vs 2.3; P<.001), though the disparity between scores was again less than in the pretest.

Comment

Our study showed that there are diagnostic disparities between lighter and darker skin types among interprofessional health care providers. Education on SOC should extend to interprofessional health care providers and other medical specialties involved in treating or triaging dermatologic diseases. A focused educational module may provide long-term improvements in diagnostic accuracy and confidence for conditions presenting in SOC. Differences in diagnostic accuracy between conditions shown in lighter and darker skin types were noted for the disease categories of infectious, cancerous, and inflammatory conditions, with the exception of conditions more frequently seen in patients with SOC. Learning resources for SOC-associated conditions are more likely to have greater representation of images depicting darker skin types.⁷ Future educational interventions may need to focus on dermatologic conditions that are not preferentially seen in patients with SOC. In our study, the pretest scores for conditions shown in darker skin were lowest among infectious and cancerous conditions. For infections, certain morphologic clues such as erythema are important for diagnosis but may be more subtle or difficult to discern in darker skin. It also is possible that providers may be less likely to suspect skin cancer in patients with SOC given that the morphologic presentation and/or anatomic site of involvement for skin cancers in SOC differs from those in lighter skin. Future educational interventions targeting disparities in diagnostic accuracy should focus on conditions that are not specifically associated with SOC.

Limitations of our study included the small number of participants, the study population came from a single institution, and a possible selection bias for providers interested in dermatology.

Conclusion

Disparities exist among interprofessional health care providers when treating conditions in patients with lighter skin compared to darker skin. An educational module for health care providers may provide long-term improvements in diagnostic accuracy and confidence for conditions presenting in patients with SOC.

Dermatologic disparities disproportionately affect patients with skin of color (SOC). Two studies assessing the diagnostic accuracy of medical students have shown disparities in diagnosing common skin conditions presenting in darker skin compared to lighter skin at early stages of training.^1,2 This knowledge gap could be attributed to the underrepresentation of SOC in dermatologic textbooks, journals, and educational curricula.^3-6 It is important for dermatologists as well as physicians in other specialties and ancillary health care workers involved in treating or triaging dermatologic diseases to recognize common skin conditions presenting in SOC. We sought to evaluate the effectiveness of a focused educational module for improving diagnostic accuracy and confidence in treating SOC among interprofessional health care providers.

Methods

Interprofessional health care providers—medical students, residents/fellows, attending physicians, advanced practice providers (APPs), and nurses practicing across various medical specialties—at The University of Texas at Austin Dell Medical School and Ascension Medical Group (both in Austin, Texas) were invited to participate in an institutional review board–exempt study involving a virtual SOC educational module from February through May 2021. The 1-hour module involved a pretest, a 15-minute lecture, an immediate posttest, and a 3-month posttest. All tests included the same 40 multiple-choice questions of 20 dermatologic conditions portrayed in lighter and darker skin types from VisualDx.com, and participants were asked to identify the condition in each photograph. Questions appeared one at a time in a randomized order, and answers could not be changed once submitted.

For analysis, the dermatologic conditions were categorized into 4 groups: cancerous, infectious, inflammatory, and SOC-associated conditions. Cancerous conditions included basal cell carcinoma, squamous cell carcinoma, and melanoma. Infectious conditions included herpes zoster, tinea corporis, tinea versicolor, staphylococcal scalded skin syndrome, and verruca vulgaris. Inflammatory conditions included acne, atopic dermatitis, pityriasis rosea, psoriasis, seborrheic dermatitis, contact dermatitis, lichen planus, and urticaria. Skin of color–associated conditions included hidradenitis suppurativa, acanthosis nigricans, keloid, and melasma. Two questions utilizing a 5-point Likert scale assessing confidence in diagnosing light and dark skin also were included.

The pre-recorded 15-minute video lecture was given by 2 dermatology residents (P.L.K. and C.P.), and the learning objectives covered morphologic differences in lighter skin and darker skin, comparisons of common dermatologic diseases in lighter skin and darker skin, diseases more commonly affecting patients with SOC, and treatment considerations for conditions affecting skin and hair in patients with SOC. Photographs from the diagnostic accuracy assessment were not reused in the lecture. Detailed explanations on morphology, diagnostic pearls, and treatment options for all conditions tested were provided to participants upon completion of the 3-month posttest.

Statistical Analysis—Test scores were compared between conditions shown in lighter and darker skin types and from the pretest to the immediate posttest and 3-month posttest. Multiple linear regression was used to assess for intervention effects on lighter and darker skin scores controlling for provider type and specialty. All tests were 2-sided with significance at P<.05. Analyses were conducted using Stata 17.

Results

One hundred participants completed the pretest and immediate posttest, 36 of whom also completed the 3-month posttest (Table). There was no significant difference in baseline characteristics between the pretest and 3-month posttest groups.

Test scores were correlated with provider type and specialty but not age, sex, or race/ethnicity. Specializing in dermatology and being a resident or attending physician were independently associated with higher test scores. Mean pretest diagnostic accuracy and confidence scores were higher for skin conditions shown in lighter skin compared with those shown in darker skin (13.6 vs 11.3 and 2.7 vs 1.9, respectively; both P<.001). Pretest diagnostic accuracy was significantly higher for skin conditions shown in lighter skin compared with darker skin for cancerous, inflammatory, and infectious conditions (72% vs 50%, 68% vs 55%, and 57% vs 47%, respectively; P<.001 for all)(Figure 1). Skin of color–associated conditions were not associated with significantly different scores for lighter skin compared with darker skin (79% vs 75%; P=.059).

Controlling for provider type and specialty, significantly improved diagnostic accuracy was seen in immediate posttest scores compared with pretest scores for conditions shown in both lighter and darker skin types (lighter: 15.2 vs 13.6; darker: 13.3 vs 11.3; both P<.001)(Figure 2). The immediate posttest demonstrated higher mean diagnostic accuracy and confidence scores for skin conditions shown in lighter skin compared with darker skin (diagnostic accuracy: 15.2 vs 13.3; confidence: 3.0 vs 2.6; both P<.001), but the disparity between scores was less than in the pretest.

Following the 3-month posttest, improvement in diagnostic accuracy was noted among both lighter and darker skin types compared with the pretest, but the difference remained significant only for conditions shown in darker skin (mean scores, 11.3 vs 13.3; P<.01). Similarly, confidence in diagnosing conditions in both lighter and darker skin improved following the immediate posttest (mean scores, 2.7 vs 3.0 and 1.9 vs 2.6; both P<.001), and this improvement remained significant for only darker skin following the 3-month posttest (mean scores, 1.9 vs 2.3; P<.001). Despite these improvements, diagnostic accuracy and confidence remained higher for skin conditions shown in lighter skin compared with darker skin (diagnostic accuracy: 14.7 vs 13.3; P<.01; confidence: 2.8 vs 2.3; P<.001), though the disparity between scores was again less than in the pretest.

Comment

Our study showed that there are diagnostic disparities between lighter and darker skin types among interprofessional health care providers. Education on SOC should extend to interprofessional health care providers and other medical specialties involved in treating or triaging dermatologic diseases. A focused educational module may provide long-term improvements in diagnostic accuracy and confidence for conditions presenting in SOC. Differences in diagnostic accuracy between conditions shown in lighter and darker skin types were noted for the disease categories of infectious, cancerous, and inflammatory conditions, with the exception of conditions more frequently seen in patients with SOC. Learning resources for SOC-associated conditions are more likely to have greater representation of images depicting darker skin types.⁷ Future educational interventions may need to focus on dermatologic conditions that are not preferentially seen in patients with SOC. In our study, the pretest scores for conditions shown in darker skin were lowest among infectious and cancerous conditions. For infections, certain morphologic clues such as erythema are important for diagnosis but may be more subtle or difficult to discern in darker skin. It also is possible that providers may be less likely to suspect skin cancer in patients with SOC given that the morphologic presentation and/or anatomic site of involvement for skin cancers in SOC differs from those in lighter skin. Future educational interventions targeting disparities in diagnostic accuracy should focus on conditions that are not specifically associated with SOC.

Limitations of our study included the small number of participants, the study population came from a single institution, and a possible selection bias for providers interested in dermatology.

Conclusion

Disparities exist among interprofessional health care providers when treating conditions in patients with lighter skin compared to darker skin. An educational module for health care providers may provide long-term improvements in diagnostic accuracy and confidence for conditions presenting in patients with SOC.

Dermatologic disparities disproportionately affect patients with skin of color (SOC). Two studies assessing the diagnostic accuracy of medical students have shown disparities in diagnosing common skin conditions presenting in darker skin compared to lighter skin at early stages of training.^1,2 This knowledge gap could be attributed to the underrepresentation of SOC in dermatologic textbooks, journals, and educational curricula.^3-6 It is important for dermatologists as well as physicians in other specialties and ancillary health care workers involved in treating or triaging dermatologic diseases to recognize common skin conditions presenting in SOC. We sought to evaluate the effectiveness of a focused educational module for improving diagnostic accuracy and confidence in treating SOC among interprofessional health care providers.

Methods

Interprofessional health care providers—medical students, residents/fellows, attending physicians, advanced practice providers (APPs), and nurses practicing across various medical specialties—at The University of Texas at Austin Dell Medical School and Ascension Medical Group (both in Austin, Texas) were invited to participate in an institutional review board–exempt study involving a virtual SOC educational module from February through May 2021. The 1-hour module involved a pretest, a 15-minute lecture, an immediate posttest, and a 3-month posttest. All tests included the same 40 multiple-choice questions of 20 dermatologic conditions portrayed in lighter and darker skin types from VisualDx.com, and participants were asked to identify the condition in each photograph. Questions appeared one at a time in a randomized order, and answers could not be changed once submitted.

For analysis, the dermatologic conditions were categorized into 4 groups: cancerous, infectious, inflammatory, and SOC-associated conditions. Cancerous conditions included basal cell carcinoma, squamous cell carcinoma, and melanoma. Infectious conditions included herpes zoster, tinea corporis, tinea versicolor, staphylococcal scalded skin syndrome, and verruca vulgaris. Inflammatory conditions included acne, atopic dermatitis, pityriasis rosea, psoriasis, seborrheic dermatitis, contact dermatitis, lichen planus, and urticaria. Skin of color–associated conditions included hidradenitis suppurativa, acanthosis nigricans, keloid, and melasma. Two questions utilizing a 5-point Likert scale assessing confidence in diagnosing light and dark skin also were included.

The pre-recorded 15-minute video lecture was given by 2 dermatology residents (P.L.K. and C.P.), and the learning objectives covered morphologic differences in lighter skin and darker skin, comparisons of common dermatologic diseases in lighter skin and darker skin, diseases more commonly affecting patients with SOC, and treatment considerations for conditions affecting skin and hair in patients with SOC. Photographs from the diagnostic accuracy assessment were not reused in the lecture. Detailed explanations on morphology, diagnostic pearls, and treatment options for all conditions tested were provided to participants upon completion of the 3-month posttest.

Statistical Analysis—Test scores were compared between conditions shown in lighter and darker skin types and from the pretest to the immediate posttest and 3-month posttest. Multiple linear regression was used to assess for intervention effects on lighter and darker skin scores controlling for provider type and specialty. All tests were 2-sided with significance at P<.05. Analyses were conducted using Stata 17.

Results

One hundred participants completed the pretest and immediate posttest, 36 of whom also completed the 3-month posttest (Table). There was no significant difference in baseline characteristics between the pretest and 3-month posttest groups.

Test scores were correlated with provider type and specialty but not age, sex, or race/ethnicity. Specializing in dermatology and being a resident or attending physician were independently associated with higher test scores. Mean pretest diagnostic accuracy and confidence scores were higher for skin conditions shown in lighter skin compared with those shown in darker skin (13.6 vs 11.3 and 2.7 vs 1.9, respectively; both P<.001). Pretest diagnostic accuracy was significantly higher for skin conditions shown in lighter skin compared with darker skin for cancerous, inflammatory, and infectious conditions (72% vs 50%, 68% vs 55%, and 57% vs 47%, respectively; P<.001 for all)(Figure 1). Skin of color–associated conditions were not associated with significantly different scores for lighter skin compared with darker skin (79% vs 75%; P=.059).

Controlling for provider type and specialty, significantly improved diagnostic accuracy was seen in immediate posttest scores compared with pretest scores for conditions shown in both lighter and darker skin types (lighter: 15.2 vs 13.6; darker: 13.3 vs 11.3; both P<.001)(Figure 2). The immediate posttest demonstrated higher mean diagnostic accuracy and confidence scores for skin conditions shown in lighter skin compared with darker skin (diagnostic accuracy: 15.2 vs 13.3; confidence: 3.0 vs 2.6; both P<.001), but the disparity between scores was less than in the pretest.

Following the 3-month posttest, improvement in diagnostic accuracy was noted among both lighter and darker skin types compared with the pretest, but the difference remained significant only for conditions shown in darker skin (mean scores, 11.3 vs 13.3; P<.01). Similarly, confidence in diagnosing conditions in both lighter and darker skin improved following the immediate posttest (mean scores, 2.7 vs 3.0 and 1.9 vs 2.6; both P<.001), and this improvement remained significant for only darker skin following the 3-month posttest (mean scores, 1.9 vs 2.3; P<.001). Despite these improvements, diagnostic accuracy and confidence remained higher for skin conditions shown in lighter skin compared with darker skin (diagnostic accuracy: 14.7 vs 13.3; P<.01; confidence: 2.8 vs 2.3; P<.001), though the disparity between scores was again less than in the pretest.

Comment

Our study showed that there are diagnostic disparities between lighter and darker skin types among interprofessional health care providers. Education on SOC should extend to interprofessional health care providers and other medical specialties involved in treating or triaging dermatologic diseases. A focused educational module may provide long-term improvements in diagnostic accuracy and confidence for conditions presenting in SOC. Differences in diagnostic accuracy between conditions shown in lighter and darker skin types were noted for the disease categories of infectious, cancerous, and inflammatory conditions, with the exception of conditions more frequently seen in patients with SOC. Learning resources for SOC-associated conditions are more likely to have greater representation of images depicting darker skin types.⁷ Future educational interventions may need to focus on dermatologic conditions that are not preferentially seen in patients with SOC. In our study, the pretest scores for conditions shown in darker skin were lowest among infectious and cancerous conditions. For infections, certain morphologic clues such as erythema are important for diagnosis but may be more subtle or difficult to discern in darker skin. It also is possible that providers may be less likely to suspect skin cancer in patients with SOC given that the morphologic presentation and/or anatomic site of involvement for skin cancers in SOC differs from those in lighter skin. Future educational interventions targeting disparities in diagnostic accuracy should focus on conditions that are not specifically associated with SOC.

Limitations of our study included the small number of participants, the study population came from a single institution, and a possible selection bias for providers interested in dermatology.

Conclusion

Disparities exist among interprofessional health care providers when treating conditions in patients with lighter skin compared to darker skin. An educational module for health care providers may provide long-term improvements in diagnostic accuracy and confidence for conditions presenting in patients with SOC.