Quiz

The Diagnosis: Atrophic Pityriasis Versicolor

Pityriasis versicolor lesions accompanied by skin atrophy were first reported by De Graciansky and Mery¹ in 1971. Since then, few reports have been described and it remains a rare condition.^2-5 It manifests with oval to round, ivory-colored lesions with a typically depressed and sometimes finely pleated surface.³ The pathogenesis of the skin atrophy remains controversial. In some of the cases described, the onset of atrophy was related to long-term use of topical steroids.¹ This fact as well as impaired barrier function due to fungal infection may explain the atrophy occurring only in the pityriasis versicolor lesions.^2,6,7 Some authors call this disease “pityriasis versicolor pseudoatrophicans.”⁷ However, case reports have been described without use of topical corticosteroids. Crowson and Magro⁸ maintained that skin atrophy in these cases may occur due to mechanisms of delayed-type hypersensitivity and coined the term atrophying pityriasis versicolor as a variant of this disease. Our patient did not report prior use of topical corticosteroids.

The differential diagnosis consists of other diseases that cause skin atrophy, such as collagen vascular diseases including anetoderma, morphea or atrophoderma, lupus erythematosus, dermatomyositis, and poikilodermatous T-cell dyscrasia; parapsoriasis or mycosis fungoides; sarcoidosis; cutis laxa; acrodermatitis chronica atrophicans; necrobiosis lipoidica; and atrophy due to intralesional steroid therapy.^2,3,6-8 Histologic examination helps to achieve proper diagnosis. In our patient, cutaneous biopsy showed the presence of multiple short hyphae and spores in the horny layer with hematoxylin and eosin as well as periodic acid–Schiff stains, with typical “spaghetti and meatballs” appearance. Partial atrophy of the epidermis was observed with flattening of the epidermic ridges. A comparison with the normal areas could not be made because the biopsy was taken from cutaneous lesions without areas of uninvolved skin (Figure).

Treatment of this variant does not differ from conventional therapies for pityriasis versicolor, except that a longer treatment period might be required. Atrophy usually disappears, showing that atrophic pityriasis versicolor has a relatively good prognosis compared with other diseases that cause skin atrophy.²

Our patient was treated with ketoconazole gel 2% once daily for 3 weeks with complete resolution of the lesions and no evidence of atrophy.

The Diagnosis: Atrophic Pityriasis Versicolor

Pityriasis versicolor lesions accompanied by skin atrophy were first reported by De Graciansky and Mery¹ in 1971. Since then, few reports have been described and it remains a rare condition.^2-5 It manifests with oval to round, ivory-colored lesions with a typically depressed and sometimes finely pleated surface.³ The pathogenesis of the skin atrophy remains controversial. In some of the cases described, the onset of atrophy was related to long-term use of topical steroids.¹ This fact as well as impaired barrier function due to fungal infection may explain the atrophy occurring only in the pityriasis versicolor lesions.^2,6,7 Some authors call this disease “pityriasis versicolor pseudoatrophicans.”⁷ However, case reports have been described without use of topical corticosteroids. Crowson and Magro⁸ maintained that skin atrophy in these cases may occur due to mechanisms of delayed-type hypersensitivity and coined the term atrophying pityriasis versicolor as a variant of this disease. Our patient did not report prior use of topical corticosteroids.

The differential diagnosis consists of other diseases that cause skin atrophy, such as collagen vascular diseases including anetoderma, morphea or atrophoderma, lupus erythematosus, dermatomyositis, and poikilodermatous T-cell dyscrasia; parapsoriasis or mycosis fungoides; sarcoidosis; cutis laxa; acrodermatitis chronica atrophicans; necrobiosis lipoidica; and atrophy due to intralesional steroid therapy.^2,3,6-8 Histologic examination helps to achieve proper diagnosis. In our patient, cutaneous biopsy showed the presence of multiple short hyphae and spores in the horny layer with hematoxylin and eosin as well as periodic acid–Schiff stains, with typical “spaghetti and meatballs” appearance. Partial atrophy of the epidermis was observed with flattening of the epidermic ridges. A comparison with the normal areas could not be made because the biopsy was taken from cutaneous lesions without areas of uninvolved skin (Figure).

Treatment of this variant does not differ from conventional therapies for pityriasis versicolor, except that a longer treatment period might be required. Atrophy usually disappears, showing that atrophic pityriasis versicolor has a relatively good prognosis compared with other diseases that cause skin atrophy.²

Our patient was treated with ketoconazole gel 2% once daily for 3 weeks with complete resolution of the lesions and no evidence of atrophy.

The Diagnosis: Atrophic Pityriasis Versicolor

Pityriasis versicolor lesions accompanied by skin atrophy were first reported by De Graciansky and Mery¹ in 1971. Since then, few reports have been described and it remains a rare condition.^2-5 It manifests with oval to round, ivory-colored lesions with a typically depressed and sometimes finely pleated surface.³ The pathogenesis of the skin atrophy remains controversial. In some of the cases described, the onset of atrophy was related to long-term use of topical steroids.¹ This fact as well as impaired barrier function due to fungal infection may explain the atrophy occurring only in the pityriasis versicolor lesions.^2,6,7 Some authors call this disease “pityriasis versicolor pseudoatrophicans.”⁷ However, case reports have been described without use of topical corticosteroids. Crowson and Magro⁸ maintained that skin atrophy in these cases may occur due to mechanisms of delayed-type hypersensitivity and coined the term atrophying pityriasis versicolor as a variant of this disease. Our patient did not report prior use of topical corticosteroids.

The differential diagnosis consists of other diseases that cause skin atrophy, such as collagen vascular diseases including anetoderma, morphea or atrophoderma, lupus erythematosus, dermatomyositis, and poikilodermatous T-cell dyscrasia; parapsoriasis or mycosis fungoides; sarcoidosis; cutis laxa; acrodermatitis chronica atrophicans; necrobiosis lipoidica; and atrophy due to intralesional steroid therapy.^2,3,6-8 Histologic examination helps to achieve proper diagnosis. In our patient, cutaneous biopsy showed the presence of multiple short hyphae and spores in the horny layer with hematoxylin and eosin as well as periodic acid–Schiff stains, with typical “spaghetti and meatballs” appearance. Partial atrophy of the epidermis was observed with flattening of the epidermic ridges. A comparison with the normal areas could not be made because the biopsy was taken from cutaneous lesions without areas of uninvolved skin (Figure).

Treatment of this variant does not differ from conventional therapies for pityriasis versicolor, except that a longer treatment period might be required. Atrophy usually disappears, showing that atrophic pityriasis versicolor has a relatively good prognosis compared with other diseases that cause skin atrophy.²

Our patient was treated with ketoconazole gel 2% once daily for 3 weeks with complete resolution of the lesions and no evidence of atrophy.

The Diagnosis: Cutaneous Leishmaniasis

On examination, the patient had multiple punched-out ulcers with indurated borders and surrounding erythema arranged in a sporotrichoid pattern from the left forearm to the left lateral chest (Figure 1).

Figure 1. Sporotrichoid spread of ulcers from the distal forearm to the lateral chest.

Bacterial culture of a tissue specimen was negative, and tissue fungal culture failed to grow any organisms. Serological studies included a complete blood cell count with differential, a chemistry panel, and liver function tests, which were all unremarkable. Coccidioidomycosis and human immunodefi-ciency virus antibodies were negative. A 4-mm punch biopsy was obtained and sent to the Armed Forces Institute of Pathology for review. Histopathologic examination revealed marked inflammation with ill-formed noncaseating granulomas and focal ulceration, necrosis in the deep dermis, and both intra-cellular and extracellular amastigotes within areas of necrosis (Figures 2 and 3).

Figure 2. Mixed dermal inflammation with ill-formed, noncaseating granulomas (H&E, original magnification ×100).

Figure 3. Multiple intracellular amastigotes are highlighted by the special stain, appearing as tiny “granules” in the cytoplasm of histiocytes (Giemsa, original magnification ×400).

The rise in the number of cases of cutaneous leishmaniasis in the United States, particularly in the veteran population, can be attributed to the recent conflicts in the Middle East and Afghanistan. Infection with Leishmania species can result in a variety of clinical presentations, ranging from localized, self-limited cutaneous lesions to a life-threatening infection with visceral involvement.¹ Additionally, the host immune response is variable. This variation in clinical presentation and disease progression explains why there is no single best treatment identified for leishmaniasis to date.

The clinical pattern of spread along the lymphatics in this patient is unique. The differential diagnosis of lesions with sporotrichoid spread includes Mycobacterium marinum and other atypical mycobacterial infections, Sporothrix schenckii, nocardiosis, leishmaniasis, coccidioidomycosis, tularemia, cat scratch disease, anthrax, chromoblastomycosis, pyogenic bacteria, and other fungal or bacterial infections. With such a broad differential diagnosis, histologic confirmation is paramount.

The most widely used pharmacotherapy for leishmaniasis is with pentavalent antimony compounds, which have been studied in randomized controlled trials for leishmaniasis more than any other drug.² These antimony compounds are associated with a large spectrum of clinical adverse events, and there is increasing evidence for emerging parasite resistance to the antimonies.^3-5 Historically, amphotericin B was considered a second-line treatment of leishmaniasis due to its systemic toxicity.⁶ However, this treatment has come back into favor due to its newer, more tolerable, lipid-associated formulation.

Our patient was treated with intravenous liposomal amphotericin B at a dosage of 3 mg/kg daily for days 1 to 5, then again on days 14 and 21. He tolerated the therapeutic regimen without difficulty or adverse effects. The ulcers eventually became smaller and ceased to weep, fully healing over a course of several months.

The Diagnosis: Cutaneous Leishmaniasis

On examination, the patient had multiple punched-out ulcers with indurated borders and surrounding erythema arranged in a sporotrichoid pattern from the left forearm to the left lateral chest (Figure 1).

Figure 1. Sporotrichoid spread of ulcers from the distal forearm to the lateral chest.

Bacterial culture of a tissue specimen was negative, and tissue fungal culture failed to grow any organisms. Serological studies included a complete blood cell count with differential, a chemistry panel, and liver function tests, which were all unremarkable. Coccidioidomycosis and human immunodefi-ciency virus antibodies were negative. A 4-mm punch biopsy was obtained and sent to the Armed Forces Institute of Pathology for review. Histopathologic examination revealed marked inflammation with ill-formed noncaseating granulomas and focal ulceration, necrosis in the deep dermis, and both intra-cellular and extracellular amastigotes within areas of necrosis (Figures 2 and 3).

Figure 2. Mixed dermal inflammation with ill-formed, noncaseating granulomas (H&E, original magnification ×100).

Figure 3. Multiple intracellular amastigotes are highlighted by the special stain, appearing as tiny “granules” in the cytoplasm of histiocytes (Giemsa, original magnification ×400).

The rise in the number of cases of cutaneous leishmaniasis in the United States, particularly in the veteran population, can be attributed to the recent conflicts in the Middle East and Afghanistan. Infection with Leishmania species can result in a variety of clinical presentations, ranging from localized, self-limited cutaneous lesions to a life-threatening infection with visceral involvement.¹ Additionally, the host immune response is variable. This variation in clinical presentation and disease progression explains why there is no single best treatment identified for leishmaniasis to date.

The clinical pattern of spread along the lymphatics in this patient is unique. The differential diagnosis of lesions with sporotrichoid spread includes Mycobacterium marinum and other atypical mycobacterial infections, Sporothrix schenckii, nocardiosis, leishmaniasis, coccidioidomycosis, tularemia, cat scratch disease, anthrax, chromoblastomycosis, pyogenic bacteria, and other fungal or bacterial infections. With such a broad differential diagnosis, histologic confirmation is paramount.

The most widely used pharmacotherapy for leishmaniasis is with pentavalent antimony compounds, which have been studied in randomized controlled trials for leishmaniasis more than any other drug.² These antimony compounds are associated with a large spectrum of clinical adverse events, and there is increasing evidence for emerging parasite resistance to the antimonies.^3-5 Historically, amphotericin B was considered a second-line treatment of leishmaniasis due to its systemic toxicity.⁶ However, this treatment has come back into favor due to its newer, more tolerable, lipid-associated formulation.

Our patient was treated with intravenous liposomal amphotericin B at a dosage of 3 mg/kg daily for days 1 to 5, then again on days 14 and 21. He tolerated the therapeutic regimen without difficulty or adverse effects. The ulcers eventually became smaller and ceased to weep, fully healing over a course of several months.

The Diagnosis: Cutaneous Leishmaniasis

On examination, the patient had multiple punched-out ulcers with indurated borders and surrounding erythema arranged in a sporotrichoid pattern from the left forearm to the left lateral chest (Figure 1).

Figure 1. Sporotrichoid spread of ulcers from the distal forearm to the lateral chest.

Bacterial culture of a tissue specimen was negative, and tissue fungal culture failed to grow any organisms. Serological studies included a complete blood cell count with differential, a chemistry panel, and liver function tests, which were all unremarkable. Coccidioidomycosis and human immunodefi-ciency virus antibodies were negative. A 4-mm punch biopsy was obtained and sent to the Armed Forces Institute of Pathology for review. Histopathologic examination revealed marked inflammation with ill-formed noncaseating granulomas and focal ulceration, necrosis in the deep dermis, and both intra-cellular and extracellular amastigotes within areas of necrosis (Figures 2 and 3).

Figure 2. Mixed dermal inflammation with ill-formed, noncaseating granulomas (H&E, original magnification ×100).

Figure 3. Multiple intracellular amastigotes are highlighted by the special stain, appearing as tiny “granules” in the cytoplasm of histiocytes (Giemsa, original magnification ×400).

The rise in the number of cases of cutaneous leishmaniasis in the United States, particularly in the veteran population, can be attributed to the recent conflicts in the Middle East and Afghanistan. Infection with Leishmania species can result in a variety of clinical presentations, ranging from localized, self-limited cutaneous lesions to a life-threatening infection with visceral involvement.¹ Additionally, the host immune response is variable. This variation in clinical presentation and disease progression explains why there is no single best treatment identified for leishmaniasis to date.

The clinical pattern of spread along the lymphatics in this patient is unique. The differential diagnosis of lesions with sporotrichoid spread includes Mycobacterium marinum and other atypical mycobacterial infections, Sporothrix schenckii, nocardiosis, leishmaniasis, coccidioidomycosis, tularemia, cat scratch disease, anthrax, chromoblastomycosis, pyogenic bacteria, and other fungal or bacterial infections. With such a broad differential diagnosis, histologic confirmation is paramount.

The most widely used pharmacotherapy for leishmaniasis is with pentavalent antimony compounds, which have been studied in randomized controlled trials for leishmaniasis more than any other drug.² These antimony compounds are associated with a large spectrum of clinical adverse events, and there is increasing evidence for emerging parasite resistance to the antimonies.^3-5 Historically, amphotericin B was considered a second-line treatment of leishmaniasis due to its systemic toxicity.⁶ However, this treatment has come back into favor due to its newer, more tolerable, lipid-associated formulation.

Our patient was treated with intravenous liposomal amphotericin B at a dosage of 3 mg/kg daily for days 1 to 5, then again on days 14 and 21. He tolerated the therapeutic regimen without difficulty or adverse effects. The ulcers eventually became smaller and ceased to weep, fully healing over a course of several months.

The Diagnosis: D-Penicillamine–Induced Elastosis Perforans Serpiginosa

A 27-year-old woman was referred to our clinic by her rheumatologist with a 2×5-cm annular plaque on the right side of the lateral neck along with nummular plaques on the left side of 1 year’s duration (Figure 1). Her medical history was notable for systemic sclerosis that had been treated with oral D-penicillamine (750 mg daily) for the last 10 years. Histopathologic examination of the skin biopsy specimen revealed an epidermis with perforating channels of elastic fibers admixed with collagen (Figure 2). Verhoeff–van Gieson elastin stain highlighted “bramble bush or lumpy-bumpy” appearance of elastic fibers in the dermis (Figure 3), which confirmed the diagnosis of D-penicillamine–induced elastosis perforans serpiginosa (EPS).

Figure 1. Nummular plaques on the left side of the lateral neck.

Figure 2. A punch biopsy specimen showed an epidermis with perforating channels of elastic fibers admixed with collagen (H&E, original magnification ×10).

Figure 3. Verhoeff–van Gieson elastin stain highlighted “bramble bush or lumpy-bumpy” appearance of elastic fibers in the dermis (original magnification ×40).

Elastosis perforans serpiginosa is a rare entity that may present in many different settings. Some associated genetic conditions include Down syndrome, pseudoxanthoma elasticum, Marfan syndrome, Ehlers-Danlos syndrome, acrogeria, osteogenesis imperfecta, Rothmund-Thomson syndrome, and moya-moya disease. Elastosis perforans serpiginosa also may be inherited in rare cases in an autosomal-dominant pattern.¹ There are solitary reports of EPS in the setting of renal disease, morphea, and systemic sclerosis. Most cases of EPS are iatrogenically acquired. As first reported in 1973, long-term D-penicillamine therapy for Wilson disease has been classically associated with the rare development of EPS.² Putative mechanisms include copper chelation by D-penicillamine in the setting of altered copper homeostasis in Wilson disease and subsequent inhibition of elastic fiber cross-linking by copper-dependent lysyl oxidase. Another proposed mechanism is the direct inhibition of collagen cross-linking by D-penicillamine resulting in abnormal elastic fiber maturation.³ Outside of the context of Wilson disease, D-penicillamine–induced EPS also has developed during the treatment of juvenile rheumatoid arthritis and cystinuria.⁴ Our patient withsystemic sclerosis also exemplifies the possibility of developing EPS from long-term D-penicillamine therapy even in the absence of coexisting Wilson disease.

Elastosis perforans serpiginosa lesions classically present as asymptomatic, serpiginously arranged, hyperkeratotic papules, nodules, and annular plaques in young adults and children. Lesions usually present on the neck, though other locations have been described. Histologically, transepidermal elimination of elastic fibers, degenerated keratinocytes, and collagen is seen in the background of a foreign-body reaction with  hematoxylin and eosin stain. Elastin stains show increased thickened elastic fibers in the dermis underlying the perforation. The histology of D-penicillamine–induced EPS is distinctive in that the elastic fibers are arranged in a bramble bush pattern with lateral buds.

The clinical course of D-penicillamine–induced EPS is variable, ranging from slow to no resolution after drug discontinuation, with residual scarring, atrophy, and concern for systemic elastosis. Adjunctive therapies include oral and topical retinoids, cryotherapy, imiquimod, and CO₂ laser.⁵ For our patient, tazarotene gel 0.1% was recommended, but the patient became pregnant soon after the diagnosis was made. Despite being untreated, her lesions have remarkably improved during her pregnancy.

The Diagnosis: D-Penicillamine–Induced Elastosis Perforans Serpiginosa

A 27-year-old woman was referred to our clinic by her rheumatologist with a 2×5-cm annular plaque on the right side of the lateral neck along with nummular plaques on the left side of 1 year’s duration (Figure 1). Her medical history was notable for systemic sclerosis that had been treated with oral D-penicillamine (750 mg daily) for the last 10 years. Histopathologic examination of the skin biopsy specimen revealed an epidermis with perforating channels of elastic fibers admixed with collagen (Figure 2). Verhoeff–van Gieson elastin stain highlighted “bramble bush or lumpy-bumpy” appearance of elastic fibers in the dermis (Figure 3), which confirmed the diagnosis of D-penicillamine–induced elastosis perforans serpiginosa (EPS).

Figure 1. Nummular plaques on the left side of the lateral neck.

Figure 2. A punch biopsy specimen showed an epidermis with perforating channels of elastic fibers admixed with collagen (H&E, original magnification ×10).

Figure 3. Verhoeff–van Gieson elastin stain highlighted “bramble bush or lumpy-bumpy” appearance of elastic fibers in the dermis (original magnification ×40).

Elastosis perforans serpiginosa is a rare entity that may present in many different settings. Some associated genetic conditions include Down syndrome, pseudoxanthoma elasticum, Marfan syndrome, Ehlers-Danlos syndrome, acrogeria, osteogenesis imperfecta, Rothmund-Thomson syndrome, and moya-moya disease. Elastosis perforans serpiginosa also may be inherited in rare cases in an autosomal-dominant pattern.¹ There are solitary reports of EPS in the setting of renal disease, morphea, and systemic sclerosis. Most cases of EPS are iatrogenically acquired. As first reported in 1973, long-term D-penicillamine therapy for Wilson disease has been classically associated with the rare development of EPS.² Putative mechanisms include copper chelation by D-penicillamine in the setting of altered copper homeostasis in Wilson disease and subsequent inhibition of elastic fiber cross-linking by copper-dependent lysyl oxidase. Another proposed mechanism is the direct inhibition of collagen cross-linking by D-penicillamine resulting in abnormal elastic fiber maturation.³ Outside of the context of Wilson disease, D-penicillamine–induced EPS also has developed during the treatment of juvenile rheumatoid arthritis and cystinuria.⁴ Our patient withsystemic sclerosis also exemplifies the possibility of developing EPS from long-term D-penicillamine therapy even in the absence of coexisting Wilson disease.

Elastosis perforans serpiginosa lesions classically present as asymptomatic, serpiginously arranged, hyperkeratotic papules, nodules, and annular plaques in young adults and children. Lesions usually present on the neck, though other locations have been described. Histologically, transepidermal elimination of elastic fibers, degenerated keratinocytes, and collagen is seen in the background of a foreign-body reaction with  hematoxylin and eosin stain. Elastin stains show increased thickened elastic fibers in the dermis underlying the perforation. The histology of D-penicillamine–induced EPS is distinctive in that the elastic fibers are arranged in a bramble bush pattern with lateral buds.

The clinical course of D-penicillamine–induced EPS is variable, ranging from slow to no resolution after drug discontinuation, with residual scarring, atrophy, and concern for systemic elastosis. Adjunctive therapies include oral and topical retinoids, cryotherapy, imiquimod, and CO₂ laser.⁵ For our patient, tazarotene gel 0.1% was recommended, but the patient became pregnant soon after the diagnosis was made. Despite being untreated, her lesions have remarkably improved during her pregnancy.

The Diagnosis: D-Penicillamine–Induced Elastosis Perforans Serpiginosa

A 27-year-old woman was referred to our clinic by her rheumatologist with a 2×5-cm annular plaque on the right side of the lateral neck along with nummular plaques on the left side of 1 year’s duration (Figure 1). Her medical history was notable for systemic sclerosis that had been treated with oral D-penicillamine (750 mg daily) for the last 10 years. Histopathologic examination of the skin biopsy specimen revealed an epidermis with perforating channels of elastic fibers admixed with collagen (Figure 2). Verhoeff–van Gieson elastin stain highlighted “bramble bush or lumpy-bumpy” appearance of elastic fibers in the dermis (Figure 3), which confirmed the diagnosis of D-penicillamine–induced elastosis perforans serpiginosa (EPS).

Figure 1. Nummular plaques on the left side of the lateral neck.

Figure 2. A punch biopsy specimen showed an epidermis with perforating channels of elastic fibers admixed with collagen (H&E, original magnification ×10).

Figure 3. Verhoeff–van Gieson elastin stain highlighted “bramble bush or lumpy-bumpy” appearance of elastic fibers in the dermis (original magnification ×40).

Elastosis perforans serpiginosa is a rare entity that may present in many different settings. Some associated genetic conditions include Down syndrome, pseudoxanthoma elasticum, Marfan syndrome, Ehlers-Danlos syndrome, acrogeria, osteogenesis imperfecta, Rothmund-Thomson syndrome, and moya-moya disease. Elastosis perforans serpiginosa also may be inherited in rare cases in an autosomal-dominant pattern.¹ There are solitary reports of EPS in the setting of renal disease, morphea, and systemic sclerosis. Most cases of EPS are iatrogenically acquired. As first reported in 1973, long-term D-penicillamine therapy for Wilson disease has been classically associated with the rare development of EPS.² Putative mechanisms include copper chelation by D-penicillamine in the setting of altered copper homeostasis in Wilson disease and subsequent inhibition of elastic fiber cross-linking by copper-dependent lysyl oxidase. Another proposed mechanism is the direct inhibition of collagen cross-linking by D-penicillamine resulting in abnormal elastic fiber maturation.³ Outside of the context of Wilson disease, D-penicillamine–induced EPS also has developed during the treatment of juvenile rheumatoid arthritis and cystinuria.⁴ Our patient withsystemic sclerosis also exemplifies the possibility of developing EPS from long-term D-penicillamine therapy even in the absence of coexisting Wilson disease.

Elastosis perforans serpiginosa lesions classically present as asymptomatic, serpiginously arranged, hyperkeratotic papules, nodules, and annular plaques in young adults and children. Lesions usually present on the neck, though other locations have been described. Histologically, transepidermal elimination of elastic fibers, degenerated keratinocytes, and collagen is seen in the background of a foreign-body reaction with  hematoxylin and eosin stain. Elastin stains show increased thickened elastic fibers in the dermis underlying the perforation. The histology of D-penicillamine–induced EPS is distinctive in that the elastic fibers are arranged in a bramble bush pattern with lateral buds.

The clinical course of D-penicillamine–induced EPS is variable, ranging from slow to no resolution after drug discontinuation, with residual scarring, atrophy, and concern for systemic elastosis. Adjunctive therapies include oral and topical retinoids, cryotherapy, imiquimod, and CO₂ laser.⁵ For our patient, tazarotene gel 0.1% was recommended, but the patient became pregnant soon after the diagnosis was made. Despite being untreated, her lesions have remarkably improved during her pregnancy.

The Diagnosis: Lues Maligna

Biopsy revealed dense nodular aggregates of lymphocytes, histiocytes, and abundant plasma cells in both the superficial and deep dermis (Figure 1). There were perivascular and periadnexal aggregates of lymphocytes, histiocytes, and numerous plasma cells (Figure 2). Special stains for organisms, including Warthin-Starry silver, Giemsa, acid-fast bacilli, Gomori methenamine-silver, and Brown-Brenn stains were negative. Immunoperoxidase stain for Treponema pallidum also was negative. The patient’s rapid plasma reagin titer at the time of the fourth biopsy was 1:256, and appropriate treatment with penicillin resulted in complete clearance of the lesions in 3 to 4 weeks.

Figure 1. Superficial and deep perivascular and periadnexal lymphohistiocytic infiltrate (H&E, original magnification ×2).

Figure 2. Perivascular and periadnexal aggregates of lymphocytes, histiocytes, and numerous plasma cells (H&E, original magnification ×20).

Syphilis is caused by T pallidum. Three stages typically are identified in immunocompetent hosts: primary, secondary, and tertiary syphilis. Immunocompromised patients with human immunodeficiency virus (HIV) infection may have unusual presentations.

Lues maligna is used to describe a rare noduloulcerative form of secondary syphilis.¹ It was first described in 1859² and has been associated with other disorders such as diabetes mellitus³ and chronic alcoholism.⁴ Patients usually are gravely ill and develop polymorphic ulcerating lesions. Facial and scalp involvement are common, but patients typically do not have palmoplantar involvement in conventional presentations of secondary syphilis.

A scanning view of a punch biopsy from our patient revealed irregular acanthosis of the epidermis with long and thin rete pegs, a bandlike infiltrate at the dermoepidermal junction, and a dense superficial and deep perivascular and periadnexal infiltrate. The histologic differential diagnosis includes pyoderma gangrenosum, vasculitis, lymphoma, leishmaniasis, leprosy, yaws, and mycobacterial or fungal infections.

The Centers for Disease Control and Prevention recommends screening of all HIV-positive indivi-duals for syphilis, and all sexually active individuals with syphilis should be screened for HIV.⁵ If clinical examination and findings suggest syphilis in the presence of negative serologic testing, then direct fluorescence assay for T pallidum staining of lesions, exudates or biopsy, or dark-field microscopic examination should be performed. In our case, dark-field microscopy was not performed and serologic tests were negative at presentation. Silver stains can detect T pallidum in tissue specimens, though detection may not be possible late in the course of disease.⁶

The morphology and rapid response to treatment confirmed the diagnosis in our patient. The incidence of syphilis in HIV-positive patients has risen substantially in the last 2 decades. This case illustrates an uncommon presentation that is increasing in prevalence.

The Diagnosis: Lues Maligna

Biopsy revealed dense nodular aggregates of lymphocytes, histiocytes, and abundant plasma cells in both the superficial and deep dermis (Figure 1). There were perivascular and periadnexal aggregates of lymphocytes, histiocytes, and numerous plasma cells (Figure 2). Special stains for organisms, including Warthin-Starry silver, Giemsa, acid-fast bacilli, Gomori methenamine-silver, and Brown-Brenn stains were negative. Immunoperoxidase stain for Treponema pallidum also was negative. The patient’s rapid plasma reagin titer at the time of the fourth biopsy was 1:256, and appropriate treatment with penicillin resulted in complete clearance of the lesions in 3 to 4 weeks.

Figure 1. Superficial and deep perivascular and periadnexal lymphohistiocytic infiltrate (H&E, original magnification ×2).

Figure 2. Perivascular and periadnexal aggregates of lymphocytes, histiocytes, and numerous plasma cells (H&E, original magnification ×20).

Syphilis is caused by T pallidum. Three stages typically are identified in immunocompetent hosts: primary, secondary, and tertiary syphilis. Immunocompromised patients with human immunodeficiency virus (HIV) infection may have unusual presentations.

Lues maligna is used to describe a rare noduloulcerative form of secondary syphilis.¹ It was first described in 1859² and has been associated with other disorders such as diabetes mellitus³ and chronic alcoholism.⁴ Patients usually are gravely ill and develop polymorphic ulcerating lesions. Facial and scalp involvement are common, but patients typically do not have palmoplantar involvement in conventional presentations of secondary syphilis.

A scanning view of a punch biopsy from our patient revealed irregular acanthosis of the epidermis with long and thin rete pegs, a bandlike infiltrate at the dermoepidermal junction, and a dense superficial and deep perivascular and periadnexal infiltrate. The histologic differential diagnosis includes pyoderma gangrenosum, vasculitis, lymphoma, leishmaniasis, leprosy, yaws, and mycobacterial or fungal infections.

The Centers for Disease Control and Prevention recommends screening of all HIV-positive indivi-duals for syphilis, and all sexually active individuals with syphilis should be screened for HIV.⁵ If clinical examination and findings suggest syphilis in the presence of negative serologic testing, then direct fluorescence assay for T pallidum staining of lesions, exudates or biopsy, or dark-field microscopic examination should be performed. In our case, dark-field microscopy was not performed and serologic tests were negative at presentation. Silver stains can detect T pallidum in tissue specimens, though detection may not be possible late in the course of disease.⁶

The morphology and rapid response to treatment confirmed the diagnosis in our patient. The incidence of syphilis in HIV-positive patients has risen substantially in the last 2 decades. This case illustrates an uncommon presentation that is increasing in prevalence.

The Diagnosis: Lues Maligna

Biopsy revealed dense nodular aggregates of lymphocytes, histiocytes, and abundant plasma cells in both the superficial and deep dermis (Figure 1). There were perivascular and periadnexal aggregates of lymphocytes, histiocytes, and numerous plasma cells (Figure 2). Special stains for organisms, including Warthin-Starry silver, Giemsa, acid-fast bacilli, Gomori methenamine-silver, and Brown-Brenn stains were negative. Immunoperoxidase stain for Treponema pallidum also was negative. The patient’s rapid plasma reagin titer at the time of the fourth biopsy was 1:256, and appropriate treatment with penicillin resulted in complete clearance of the lesions in 3 to 4 weeks.

Figure 1. Superficial and deep perivascular and periadnexal lymphohistiocytic infiltrate (H&E, original magnification ×2).

Figure 2. Perivascular and periadnexal aggregates of lymphocytes, histiocytes, and numerous plasma cells (H&E, original magnification ×20).

Syphilis is caused by T pallidum. Three stages typically are identified in immunocompetent hosts: primary, secondary, and tertiary syphilis. Immunocompromised patients with human immunodeficiency virus (HIV) infection may have unusual presentations.

Lues maligna is used to describe a rare noduloulcerative form of secondary syphilis.¹ It was first described in 1859² and has been associated with other disorders such as diabetes mellitus³ and chronic alcoholism.⁴ Patients usually are gravely ill and develop polymorphic ulcerating lesions. Facial and scalp involvement are common, but patients typically do not have palmoplantar involvement in conventional presentations of secondary syphilis.

A scanning view of a punch biopsy from our patient revealed irregular acanthosis of the epidermis with long and thin rete pegs, a bandlike infiltrate at the dermoepidermal junction, and a dense superficial and deep perivascular and periadnexal infiltrate. The histologic differential diagnosis includes pyoderma gangrenosum, vasculitis, lymphoma, leishmaniasis, leprosy, yaws, and mycobacterial or fungal infections.

The Centers for Disease Control and Prevention recommends screening of all HIV-positive indivi-duals for syphilis, and all sexually active individuals with syphilis should be screened for HIV.⁵ If clinical examination and findings suggest syphilis in the presence of negative serologic testing, then direct fluorescence assay for T pallidum staining of lesions, exudates or biopsy, or dark-field microscopic examination should be performed. In our case, dark-field microscopy was not performed and serologic tests were negative at presentation. Silver stains can detect T pallidum in tissue specimens, though detection may not be possible late in the course of disease.⁶

The morphology and rapid response to treatment confirmed the diagnosis in our patient. The incidence of syphilis in HIV-positive patients has risen substantially in the last 2 decades. This case illustrates an uncommon presentation that is increasing in prevalence.

The Diagnosis: Adult-Onset Xanthogranuloma

Biopsies of the lesions on the neck (Figure 1) and back were performed. Histologic analyses revealed a diffuse dermatitis consisting of foamy histiocytes admixed with a few Touton-type giant cells in the dermis (Figure 2), which was associated with an inflammatory infiltrate of eosinophils and lymphocytes. Laboratory investigations revealed mild thrombocytopenia with a platelet count of 134×10⁹/L (reference range, 140–440×10⁹/L). Other investigations including biochemistry, lipid, serum electrophoresis, and chest radiogram were normal. A bone marrow trephine biopsy and flow cytometry were performed and were normal. Magnetic resonance imaging revealed periorbital soft-tissue masses that did not extend into the orbits.

Figure 1. Firm plaques with a yellowish tinge over the side of the neck

Figure 2. A dense infiltrate of foamy histiocytes in the dermis (A) associated with a Touton-type giant cell (arrow) and an inflammatory infiltrate consisting of eosinophils and lymphocytes (B)(both H&E, original magnifications ×200 and ×400).

Adult-onset xanthogranuloma (AXG) is a rare disease entity, usually presenting in the third to fourth decades of life. The condition typically presents as a red to yellow-brown nodular cutaneous lesion located on the scalp, face, neck, trunk, or limbs. The presentation typically consists of a solitary lesion, occurring in 70% to 89% of cases,¹ but more rarely, as in this case, lesions can be multiple or even disseminated.

Histologically, AXG presents as a dense, well-circumscribed, histiocytic infiltrate consisting of lipophages possessing foamy cytoplasm and giant cells. The presence of histiocytic giant cells differentiates AXG from xanthelasma, a clinical differential diagnosis in this case, and xanthoma. In AXG, there are 4 main types of histiocytes: xanthomatized, spindle shaped, vacuolated, and oncocytic.² They can be seen in variable proportions, together with different types of giant cells (eg, Touton, foreign body, ground glass, nonspecific). A mixed infiltrate of eosinophils, lymphocytes, plasma cells, and neutrophils also may be seen scattered throughout the lesion.²

Correlating with the clinical and histological features of xanthogranuloma, the firm plaques and nodules represent the dense dermal infiltration of histiocytes that may extend into the subcutis. The lesions demonstrate a time-dependent progression both clinically and histologically. Early lesions are comprised of a dense monomorphous nonlipid histiocytic inflammatory infiltrate, and they clinically appear more erythematous. In mature lesions, as in our patient, the infiltrate is predominantly composed of lipid-laden histiocytes with associated Touton giant cells. They appear more yellowish on clinical presentation.

Adult-onset xanthogranuloma is part of a rare heterogenous group of disorders termed adult orbital xanthogranulomatous disease, which includes 3 other syndromes: necrobiotic xanthogranuloma, adult-onset asthma and periocular xanthogranuloma, and Erdheim-Chester disease.

Necrobiotic xanthogranuloma is clinically characterized by the presence of subcutaneous lesions that ulcerate in approximately 40% to 50% of cases and is histologically characterized by necrobiosis with palisading epithelioid histiocytes. It also is systemically associated with paraproteinemia and multiple myeloma.³

Adult-onset asthma and periocular xanthogranuloma is characterized by yellowish papules and nodules predominantly over the lower eyelids that are histologically comprised of lymphoid follicles with reactive germinal centers. It is associated with asthma, which normally is severe and often occurs almost simultaneously with the periorbital lesions.⁴

There are no definite diagnostic criteria for Erdheim-Chester disease and the diagnosis is usually based on radiologic findings of osteosclerosis and histopathologic evidence of foamy histiocytic infiltration. Systemic manifestations are common with lymphohistiocytic infiltration of the heart, lungs, pericardium, bones, and intestines. Prognosis is uniformly dismal.

Based on the clinical presentation of a nonulcerative papulonodular eruption and the absence of systemic involvement including asthma, we made the diagnosis of AXG. In view of the heterogeneity among these clinical entities as well as the time-based evolution of the lesions involved, we continued to monitor the patient for 2 years and there was no development of other systemic manifestations and hematologic abnormalities.

In contrast to the more common form of juvenile-onset xanthogranuloma, the adult type is not associated with widespread visceral lesions. Hence extensive screening investigations for systemic disease generally are not necessary. Another difference is that AXG has been associated with hematologic abnormalities, including essential thrombocytosis, chronic lymphocytic leukemia, large B-cell lymphoma, and monoclonal gammopathy.^5,6 In our patient, the presence of thrombocytopenia and older age caused us to be concerned about an associated hematologic malignancy; therefore, a bone marrow biopsy and flow cytometry were performed.

Adult-onset xanthogranuloma typically is an asymptomatic and self-healing disease and therefore treatment usually is not required. Spontaneous regression of xanthogranuloma was observed to occur in 54% of cases with a median duration of 22 months,⁷ though lesions were noted to last as long as 15 years.⁸ When treatment is necessary, a combination of local and systemic corticosteroids, cytotoxic agents, and radiotherapy have been routinely used. In particular, intralesional corticosteroid therapy has been found to be efficacious in controlling symptoms and signs of AXG affecting the eyelids and orbits while avoiding the systemic side effects of other agents.⁹

Because our patient’s lesions were longstanding and disfiguring, we opted for active intervention with intralesional triamcinolone, which resulted in only a slight reduction in size of the lesions. The lesions remain largely unchanged in 2 years of follow-up.

The Diagnosis: Adult-Onset Xanthogranuloma

Biopsies of the lesions on the neck (Figure 1) and back were performed. Histologic analyses revealed a diffuse dermatitis consisting of foamy histiocytes admixed with a few Touton-type giant cells in the dermis (Figure 2), which was associated with an inflammatory infiltrate of eosinophils and lymphocytes. Laboratory investigations revealed mild thrombocytopenia with a platelet count of 134×10⁹/L (reference range, 140–440×10⁹/L). Other investigations including biochemistry, lipid, serum electrophoresis, and chest radiogram were normal. A bone marrow trephine biopsy and flow cytometry were performed and were normal. Magnetic resonance imaging revealed periorbital soft-tissue masses that did not extend into the orbits.

Figure 1. Firm plaques with a yellowish tinge over the side of the neck

Figure 2. A dense infiltrate of foamy histiocytes in the dermis (A) associated with a Touton-type giant cell (arrow) and an inflammatory infiltrate consisting of eosinophils and lymphocytes (B)(both H&E, original magnifications ×200 and ×400).

Adult-onset xanthogranuloma (AXG) is a rare disease entity, usually presenting in the third to fourth decades of life. The condition typically presents as a red to yellow-brown nodular cutaneous lesion located on the scalp, face, neck, trunk, or limbs. The presentation typically consists of a solitary lesion, occurring in 70% to 89% of cases,¹ but more rarely, as in this case, lesions can be multiple or even disseminated.

Histologically, AXG presents as a dense, well-circumscribed, histiocytic infiltrate consisting of lipophages possessing foamy cytoplasm and giant cells. The presence of histiocytic giant cells differentiates AXG from xanthelasma, a clinical differential diagnosis in this case, and xanthoma. In AXG, there are 4 main types of histiocytes: xanthomatized, spindle shaped, vacuolated, and oncocytic.² They can be seen in variable proportions, together with different types of giant cells (eg, Touton, foreign body, ground glass, nonspecific). A mixed infiltrate of eosinophils, lymphocytes, plasma cells, and neutrophils also may be seen scattered throughout the lesion.²

Correlating with the clinical and histological features of xanthogranuloma, the firm plaques and nodules represent the dense dermal infiltration of histiocytes that may extend into the subcutis. The lesions demonstrate a time-dependent progression both clinically and histologically. Early lesions are comprised of a dense monomorphous nonlipid histiocytic inflammatory infiltrate, and they clinically appear more erythematous. In mature lesions, as in our patient, the infiltrate is predominantly composed of lipid-laden histiocytes with associated Touton giant cells. They appear more yellowish on clinical presentation.

Adult-onset xanthogranuloma is part of a rare heterogenous group of disorders termed adult orbital xanthogranulomatous disease, which includes 3 other syndromes: necrobiotic xanthogranuloma, adult-onset asthma and periocular xanthogranuloma, and Erdheim-Chester disease.

Necrobiotic xanthogranuloma is clinically characterized by the presence of subcutaneous lesions that ulcerate in approximately 40% to 50% of cases and is histologically characterized by necrobiosis with palisading epithelioid histiocytes. It also is systemically associated with paraproteinemia and multiple myeloma.³

Adult-onset asthma and periocular xanthogranuloma is characterized by yellowish papules and nodules predominantly over the lower eyelids that are histologically comprised of lymphoid follicles with reactive germinal centers. It is associated with asthma, which normally is severe and often occurs almost simultaneously with the periorbital lesions.⁴

There are no definite diagnostic criteria for Erdheim-Chester disease and the diagnosis is usually based on radiologic findings of osteosclerosis and histopathologic evidence of foamy histiocytic infiltration. Systemic manifestations are common with lymphohistiocytic infiltration of the heart, lungs, pericardium, bones, and intestines. Prognosis is uniformly dismal.

Based on the clinical presentation of a nonulcerative papulonodular eruption and the absence of systemic involvement including asthma, we made the diagnosis of AXG. In view of the heterogeneity among these clinical entities as well as the time-based evolution of the lesions involved, we continued to monitor the patient for 2 years and there was no development of other systemic manifestations and hematologic abnormalities.

In contrast to the more common form of juvenile-onset xanthogranuloma, the adult type is not associated with widespread visceral lesions. Hence extensive screening investigations for systemic disease generally are not necessary. Another difference is that AXG has been associated with hematologic abnormalities, including essential thrombocytosis, chronic lymphocytic leukemia, large B-cell lymphoma, and monoclonal gammopathy.^5,6 In our patient, the presence of thrombocytopenia and older age caused us to be concerned about an associated hematologic malignancy; therefore, a bone marrow biopsy and flow cytometry were performed.

Adult-onset xanthogranuloma typically is an asymptomatic and self-healing disease and therefore treatment usually is not required. Spontaneous regression of xanthogranuloma was observed to occur in 54% of cases with a median duration of 22 months,⁷ though lesions were noted to last as long as 15 years.⁸ When treatment is necessary, a combination of local and systemic corticosteroids, cytotoxic agents, and radiotherapy have been routinely used. In particular, intralesional corticosteroid therapy has been found to be efficacious in controlling symptoms and signs of AXG affecting the eyelids and orbits while avoiding the systemic side effects of other agents.⁹

Because our patient’s lesions were longstanding and disfiguring, we opted for active intervention with intralesional triamcinolone, which resulted in only a slight reduction in size of the lesions. The lesions remain largely unchanged in 2 years of follow-up.

The Diagnosis: Adult-Onset Xanthogranuloma

Biopsies of the lesions on the neck (Figure 1) and back were performed. Histologic analyses revealed a diffuse dermatitis consisting of foamy histiocytes admixed with a few Touton-type giant cells in the dermis (Figure 2), which was associated with an inflammatory infiltrate of eosinophils and lymphocytes. Laboratory investigations revealed mild thrombocytopenia with a platelet count of 134×10⁹/L (reference range, 140–440×10⁹/L). Other investigations including biochemistry, lipid, serum electrophoresis, and chest radiogram were normal. A bone marrow trephine biopsy and flow cytometry were performed and were normal. Magnetic resonance imaging revealed periorbital soft-tissue masses that did not extend into the orbits.

Figure 1. Firm plaques with a yellowish tinge over the side of the neck

Figure 2. A dense infiltrate of foamy histiocytes in the dermis (A) associated with a Touton-type giant cell (arrow) and an inflammatory infiltrate consisting of eosinophils and lymphocytes (B)(both H&E, original magnifications ×200 and ×400).

Adult-onset xanthogranuloma (AXG) is a rare disease entity, usually presenting in the third to fourth decades of life. The condition typically presents as a red to yellow-brown nodular cutaneous lesion located on the scalp, face, neck, trunk, or limbs. The presentation typically consists of a solitary lesion, occurring in 70% to 89% of cases,¹ but more rarely, as in this case, lesions can be multiple or even disseminated.

Histologically, AXG presents as a dense, well-circumscribed, histiocytic infiltrate consisting of lipophages possessing foamy cytoplasm and giant cells. The presence of histiocytic giant cells differentiates AXG from xanthelasma, a clinical differential diagnosis in this case, and xanthoma. In AXG, there are 4 main types of histiocytes: xanthomatized, spindle shaped, vacuolated, and oncocytic.² They can be seen in variable proportions, together with different types of giant cells (eg, Touton, foreign body, ground glass, nonspecific). A mixed infiltrate of eosinophils, lymphocytes, plasma cells, and neutrophils also may be seen scattered throughout the lesion.²

Correlating with the clinical and histological features of xanthogranuloma, the firm plaques and nodules represent the dense dermal infiltration of histiocytes that may extend into the subcutis. The lesions demonstrate a time-dependent progression both clinically and histologically. Early lesions are comprised of a dense monomorphous nonlipid histiocytic inflammatory infiltrate, and they clinically appear more erythematous. In mature lesions, as in our patient, the infiltrate is predominantly composed of lipid-laden histiocytes with associated Touton giant cells. They appear more yellowish on clinical presentation.

Adult-onset xanthogranuloma is part of a rare heterogenous group of disorders termed adult orbital xanthogranulomatous disease, which includes 3 other syndromes: necrobiotic xanthogranuloma, adult-onset asthma and periocular xanthogranuloma, and Erdheim-Chester disease.

Necrobiotic xanthogranuloma is clinically characterized by the presence of subcutaneous lesions that ulcerate in approximately 40% to 50% of cases and is histologically characterized by necrobiosis with palisading epithelioid histiocytes. It also is systemically associated with paraproteinemia and multiple myeloma.³

Adult-onset asthma and periocular xanthogranuloma is characterized by yellowish papules and nodules predominantly over the lower eyelids that are histologically comprised of lymphoid follicles with reactive germinal centers. It is associated with asthma, which normally is severe and often occurs almost simultaneously with the periorbital lesions.⁴

There are no definite diagnostic criteria for Erdheim-Chester disease and the diagnosis is usually based on radiologic findings of osteosclerosis and histopathologic evidence of foamy histiocytic infiltration. Systemic manifestations are common with lymphohistiocytic infiltration of the heart, lungs, pericardium, bones, and intestines. Prognosis is uniformly dismal.

Based on the clinical presentation of a nonulcerative papulonodular eruption and the absence of systemic involvement including asthma, we made the diagnosis of AXG. In view of the heterogeneity among these clinical entities as well as the time-based evolution of the lesions involved, we continued to monitor the patient for 2 years and there was no development of other systemic manifestations and hematologic abnormalities.

In contrast to the more common form of juvenile-onset xanthogranuloma, the adult type is not associated with widespread visceral lesions. Hence extensive screening investigations for systemic disease generally are not necessary. Another difference is that AXG has been associated with hematologic abnormalities, including essential thrombocytosis, chronic lymphocytic leukemia, large B-cell lymphoma, and monoclonal gammopathy.^5,6 In our patient, the presence of thrombocytopenia and older age caused us to be concerned about an associated hematologic malignancy; therefore, a bone marrow biopsy and flow cytometry were performed.

Adult-onset xanthogranuloma typically is an asymptomatic and self-healing disease and therefore treatment usually is not required. Spontaneous regression of xanthogranuloma was observed to occur in 54% of cases with a median duration of 22 months,⁷ though lesions were noted to last as long as 15 years.⁸ When treatment is necessary, a combination of local and systemic corticosteroids, cytotoxic agents, and radiotherapy have been routinely used. In particular, intralesional corticosteroid therapy has been found to be efficacious in controlling symptoms and signs of AXG affecting the eyelids and orbits while avoiding the systemic side effects of other agents.⁹

Because our patient’s lesions were longstanding and disfiguring, we opted for active intervention with intralesional triamcinolone, which resulted in only a slight reduction in size of the lesions. The lesions remain largely unchanged in 2 years of follow-up.

The Diagnosis: Disseminated Superficial Actinic Porokeratosis

Physical examination after 7 years of tanning salon use showed a tanned white man with multiple 4- to 5-mm, discrete, round to oval, reddish brown papules on the chest, back, abdomen, arms, and legs that were rough to palpate (Figure 1), with a peripheral rim of scale seen more prominently on dermoscopy. There were no lesions on the palms or soles. A subsequent 4-mm punch biopsy was done on the right abdomen and right thigh, which showed focal thinning of the epidermis, loss of the granular layer, a discrete column of parakeratosis and a characteristic feature of a coronoid lamella in the epidermis (Figure 2). The patient received 14 narrowband UVB (NB-UVB) treatments; however, he could not continue due to transportation issues. He visited the clinic sporadically for 6 months thereafter and reportedly went to tanning salons daily. The patient was subsequently lost to follow-up.

Figure 1. Multiple 4- to 5-mm, discrete, round to oval, reddish brown papules on the abdomen (A) and leg (B) that were rough to palpate.

Disseminated porokeratosis, or disseminated superficial actinic porokeratosis (DSAP), was first described in 1967 by Chernosky and Freeman.¹ It is the most common variant of porokeratosis. Other variants include Mibelli type, porokeratosis palmaris et plantaris disseminata, punctuate porokeratosis, and linear porokeratosis.^2-4 Porokeratosis is inherited in an autosomal-dominant fashion, presenting in the third or fourth decades of life; however, most cases are sporadic.⁵ Pruritus is a common symptom and can be debilitating.⁵

Disseminated superficial actinic porokeratosis can be precipitated by excessive sun exposure, with a reported increase in lesions during summer months and resolution during the winter months.⁶ The lesions of DSAP can be experimentally induced by exposure to daily use of artificial UV sunlamps.⁷ Patients with psoriasis undergoing psoralen plus UVA and NB-UVB treatments also have been reported to trigger DSAP.⁸ A study by Neumann et al⁶ suggested that a combination of both UVB and UVA wavelengths may be most effective in inducing DSAP. Exposure to UVA and UVB light may explain an increased number of DSAP lesions in patients who excessively visit tanning salons, as the bulbs emit a combination of wavelengths with UVA in much greater amounts than UVB.

Our patient developed DSAP secondary to artificial UV light exposure from excessive tanning salon use. Medications (allopurinol and lisinopril) were initially thought to be etiologic agents for the eruption and also corroborated with histologic findings of a drug eruption on the initial biopsy. However, new lesions continued to develop even after cessation of medications and NB-UVB treatments. A subsequent biopsy and further history of daily tanning salon use confirmed the diagnosis of DSAP.

Figure 2. Biopsy revealed a discrete column of parakeratosis, a characteristic feature of a coronoid lamella in the epidermis, and moderate papillary dermis lymphocytic infiltrate (H&E, original magnification ×10).

Therapies for this condition are limited with variable degrees of success. Cryotherapy, 5-fluorouracil cream, imiquimod cream 5%, Q-switched ruby laser, diclofenac gel 3%, and acitretin for more widespread or refractory lesions have been used with partial to complete resolution of DSAP.⁹

We present this case to highlight the occurrence of DSAP secondary to UV light exposure from excessive tanning salon use.

The Diagnosis: Disseminated Superficial Actinic Porokeratosis

Physical examination after 7 years of tanning salon use showed a tanned white man with multiple 4- to 5-mm, discrete, round to oval, reddish brown papules on the chest, back, abdomen, arms, and legs that were rough to palpate (Figure 1), with a peripheral rim of scale seen more prominently on dermoscopy. There were no lesions on the palms or soles. A subsequent 4-mm punch biopsy was done on the right abdomen and right thigh, which showed focal thinning of the epidermis, loss of the granular layer, a discrete column of parakeratosis and a characteristic feature of a coronoid lamella in the epidermis (Figure 2). The patient received 14 narrowband UVB (NB-UVB) treatments; however, he could not continue due to transportation issues. He visited the clinic sporadically for 6 months thereafter and reportedly went to tanning salons daily. The patient was subsequently lost to follow-up.

Figure 1. Multiple 4- to 5-mm, discrete, round to oval, reddish brown papules on the abdomen (A) and leg (B) that were rough to palpate.

Disseminated porokeratosis, or disseminated superficial actinic porokeratosis (DSAP), was first described in 1967 by Chernosky and Freeman.¹ It is the most common variant of porokeratosis. Other variants include Mibelli type, porokeratosis palmaris et plantaris disseminata, punctuate porokeratosis, and linear porokeratosis.^2-4 Porokeratosis is inherited in an autosomal-dominant fashion, presenting in the third or fourth decades of life; however, most cases are sporadic.⁵ Pruritus is a common symptom and can be debilitating.⁵

Disseminated superficial actinic porokeratosis can be precipitated by excessive sun exposure, with a reported increase in lesions during summer months and resolution during the winter months.⁶ The lesions of DSAP can be experimentally induced by exposure to daily use of artificial UV sunlamps.⁷ Patients with psoriasis undergoing psoralen plus UVA and NB-UVB treatments also have been reported to trigger DSAP.⁸ A study by Neumann et al⁶ suggested that a combination of both UVB and UVA wavelengths may be most effective in inducing DSAP. Exposure to UVA and UVB light may explain an increased number of DSAP lesions in patients who excessively visit tanning salons, as the bulbs emit a combination of wavelengths with UVA in much greater amounts than UVB.

Our patient developed DSAP secondary to artificial UV light exposure from excessive tanning salon use. Medications (allopurinol and lisinopril) were initially thought to be etiologic agents for the eruption and also corroborated with histologic findings of a drug eruption on the initial biopsy. However, new lesions continued to develop even after cessation of medications and NB-UVB treatments. A subsequent biopsy and further history of daily tanning salon use confirmed the diagnosis of DSAP.

Figure 2. Biopsy revealed a discrete column of parakeratosis, a characteristic feature of a coronoid lamella in the epidermis, and moderate papillary dermis lymphocytic infiltrate (H&E, original magnification ×10).

Therapies for this condition are limited with variable degrees of success. Cryotherapy, 5-fluorouracil cream, imiquimod cream 5%, Q-switched ruby laser, diclofenac gel 3%, and acitretin for more widespread or refractory lesions have been used with partial to complete resolution of DSAP.⁹

We present this case to highlight the occurrence of DSAP secondary to UV light exposure from excessive tanning salon use.

The Diagnosis: Disseminated Superficial Actinic Porokeratosis

Physical examination after 7 years of tanning salon use showed a tanned white man with multiple 4- to 5-mm, discrete, round to oval, reddish brown papules on the chest, back, abdomen, arms, and legs that were rough to palpate (Figure 1), with a peripheral rim of scale seen more prominently on dermoscopy. There were no lesions on the palms or soles. A subsequent 4-mm punch biopsy was done on the right abdomen and right thigh, which showed focal thinning of the epidermis, loss of the granular layer, a discrete column of parakeratosis and a characteristic feature of a coronoid lamella in the epidermis (Figure 2). The patient received 14 narrowband UVB (NB-UVB) treatments; however, he could not continue due to transportation issues. He visited the clinic sporadically for 6 months thereafter and reportedly went to tanning salons daily. The patient was subsequently lost to follow-up.

Figure 1. Multiple 4- to 5-mm, discrete, round to oval, reddish brown papules on the abdomen (A) and leg (B) that were rough to palpate.

Disseminated porokeratosis, or disseminated superficial actinic porokeratosis (DSAP), was first described in 1967 by Chernosky and Freeman.¹ It is the most common variant of porokeratosis. Other variants include Mibelli type, porokeratosis palmaris et plantaris disseminata, punctuate porokeratosis, and linear porokeratosis.^2-4 Porokeratosis is inherited in an autosomal-dominant fashion, presenting in the third or fourth decades of life; however, most cases are sporadic.⁵ Pruritus is a common symptom and can be debilitating.⁵

Disseminated superficial actinic porokeratosis can be precipitated by excessive sun exposure, with a reported increase in lesions during summer months and resolution during the winter months.⁶ The lesions of DSAP can be experimentally induced by exposure to daily use of artificial UV sunlamps.⁷ Patients with psoriasis undergoing psoralen plus UVA and NB-UVB treatments also have been reported to trigger DSAP.⁸ A study by Neumann et al⁶ suggested that a combination of both UVB and UVA wavelengths may be most effective in inducing DSAP. Exposure to UVA and UVB light may explain an increased number of DSAP lesions in patients who excessively visit tanning salons, as the bulbs emit a combination of wavelengths with UVA in much greater amounts than UVB.

Our patient developed DSAP secondary to artificial UV light exposure from excessive tanning salon use. Medications (allopurinol and lisinopril) were initially thought to be etiologic agents for the eruption and also corroborated with histologic findings of a drug eruption on the initial biopsy. However, new lesions continued to develop even after cessation of medications and NB-UVB treatments. A subsequent biopsy and further history of daily tanning salon use confirmed the diagnosis of DSAP.

Figure 2. Biopsy revealed a discrete column of parakeratosis, a characteristic feature of a coronoid lamella in the epidermis, and moderate papillary dermis lymphocytic infiltrate (H&E, original magnification ×10).

Therapies for this condition are limited with variable degrees of success. Cryotherapy, 5-fluorouracil cream, imiquimod cream 5%, Q-switched ruby laser, diclofenac gel 3%, and acitretin for more widespread or refractory lesions have been used with partial to complete resolution of DSAP.⁹

We present this case to highlight the occurrence of DSAP secondary to UV light exposure from excessive tanning salon use.