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Epidermodysplasia verruciformis associated with HPV 10
P5929
P7029
Epidermodysplasia verruciformis associated with HPV 10 Amir Zahir, MD, George Washington University Medical Faculty Associates, Washington, DC, United States; Alison Ehrlich, MD, MHS, George Washington University Medical Faculty Associates, Washington, DC, United States; Lauren Craig, George Washington University School of Medicine and Health Sciences, Washington, DC, United States; Peter Rady, MD, PhD, University of Texas Medical School at Houston, Houston, TX, United States; Stephen Tyring, MD, PhD, University of Texas Medical School at Houston, Houston, TX, United States Case report: A 42-year-old white man presented to our clinic with a 30-year history of reddish-brown papules on the extensor surfaces of his arms and anterior legs. He was treated with liquid nitrogen 9 years ago, and the treatment resulted in central clearing of his lesions. On examination, he had erythematous papules and macules on the extensor surfaces of his forearms. No clinical sign of cutaneous carcinoma was present. He has no personal history of skin cancer or immunosuppression. The patient was adopted and does not know if he has a family history of recalcitrant warts or skin cancer. A biopsy of a papule on his left arm revealed epidermal acanthosis, hyperkeratosis, vacuolation of superficial keratinocytes in the granular layer, and mild perivascular infiltrate in the superficial dermis. These findings are consistent with verruca plana. In situ hybridization was performed to detect multiple HPV subtypes. The tissues were analyzed for HPV types 2, 5, 6, 8, 11, 16, 18, 30, 31, 33, 35, 42, 43, 44, 45, 51, 52, 68, and 70. The results were all negative. HPV typing by nested PCR for the detection of epidermodysplasia verruciformis associated HPV types using an EV-HPV primer system was negative. Single PCR using the GP5+/6+ HPV primer system revealed a putative HPV fragment. The sequencing of the isolated PCR products and the computer assisted analysis (NCBI-BLAST) of the PCR products was identified as HPV type 10 DNA. Discussion: Epidermodysplasia verruciformis (EV) is a rare, inherited dermatologic condition demonstrating an increased susceptibility to specific HPV genotypes, resulting in both benign and malignant skin lesions. Acquired EV has been described in patients with HIV or in those who are on immunosuppressive therapy. EV lesions are frequently described as pityriasis versicolorelike scaly macules, flat wart-like papules, or as verrucous and seborrhic keratosiselike papules and plaques. These lesions range in pigmentation and often amalgamate to form patches and plaques. The classic histologic finding of epidermodysplasia verruciformis is a verruca planaetype lesion with mild to moderate hyperkeratosis, acanthosis, and enlarged vacuolated keratinocytes with perinuclear halos and blue-gray cytoplasmic pallor. We discuss a patient with EV who presents with plane wartetype skin lesions associated with HPV 10, a less frequently cited causative subtype, and histologic findings that are not classic for EV.
Erythema elevatum diutinum Fabienne Robuschi-Lestouquet, MD, Complejo Hospitalario Toledo, Toledo, Spain; Blas Gomez-Dorado, MD, Complejo Hospitalario Toledo, Toledo, Spain; Cristina Cardona-Alzate, MD, Complejo Hospitalario Toledo, Toledo, Spain; Cristina Schoendorff-Ortega, MD, Complejo Hospitalario Toledo, Toledo, Spain; Elena Vera, MD, Complejo Hospitalario Toledo, Toledo, Spain; Raquel Martin, MD, Complejo Hospitalario Toledo, Toledo, Spain Erythema elevatum diutinum (EED) is considered a chronic cutaneous vasculitis We reported 2 cases. Case 1 is a 59-year-old woman who presented with a 1-year history of itchy nodules on the elbows, and since the last week bilateral asymptomatic lower limb lesions. Her medical history was remarkable for rheumatic arthritis. Physical examination revealed erythematous to skin colored papules and nodules on her both elbows and forearm. Moreover, she had bilateral reddish to purple macules and papules on her legs. Laboratory investigations revealed anemia (Hb 10, 1 g/dL, hto 32%), protein C reactive, and VSG elevated. Skin biopsy was performed of the nodule from the elbow and histopathology revealed dermal fibrosis, dense dermal infiltrate composed of lymphocytes, and neutrophils. Leukocytoclastic vasculitis (LCV) with areas of fibrinoid necrosis and neutrophils infiltrating the vessels walls was seen. A diagnosis of EED was made and the patient is without treatment. Case 2 is a 50-year-old man who presented with year and half history of papules on the extensor surfaces of extremities and occasionally arthalgia. A physical examination revealed firm erythematous to skin-colored papules on his dorsal hands, elbows, knees, ankles and on left ear. Laboratory investigations revealed anemia (Hb 10, g/dL, hto 30%), VSG elevated. Histopathologic examinations of the skin lesions from the left forearm revealed vasculitis in the small vessels of the dermis with fibrinoid deposits and extravasated red blood cells. A diagnosis of EED was made and he was treated with dapsone 100 mg daily with a good response in 4 months. EED is considered to be a chronic fibrosing leukocytoclastic vasculitis. The cause is unknown, and it is believed to be an immune complexemediated vasculitis. EED has been linked to autoimmune processes, hematologic abnormalities, and infectious disease. Autoinmune processes include rheumatoid arthritis. Clinically typical lesions are erythematous, yellow-brown, papules and nodules.The extensor surfaces of the extremities are most commonly involved. Histopathologically acute lesions show a neutrophilic pervascular infiltrate with dermal fibrin deposits and leucocytoclasis. Mature lesions show dermal fibrosis and vasculature prominence with persistent inflammation with eosinophils, histiocytes, and lymphocytes. Treatment of EED is difficult because this disorder follows a chronic and recurrent course. Dapsone is considered first-line treatment for EED. Commercial support: None identified.
Commercial support: None identified.
P6067 Erythema elevatum diutinum in a 32-year-old pregnant woman Jason Michaels, MD, MS, Mayo Clinic, Scottsdale, AZ, United States; Catherine Harmon, MD, Mayo Clinic, Scottsdale, AZ, United States; David DiCaudo, MD, Mayo Clinic, Scottsdale, AZ, United States; James Yiannias, MD, Mayo Clinic, Scottsdale, AZ, United States
Case report: We present a case of eruptive collagenomas of the skin in a 68-year-old woman. No familal association was detected or systemic disorders associated. We describe the typical clinical features and histology of this rare condition. The differential diagnosis and other types of cutaneous collagenomas are briefly discussed. A biopsy was required to confirm the excessive mature collagen that predominates in these lesions. Discussion: Connective tissue nevi are circumscribed alterations in the development of the dermis that may be congenital or become apparent in later life. They are slowly progressive and permanent. Eruptive collagenomas can be distinguished from other collagenomas by history and examination. The incidence of eruptive collagenoma is not known but all cases described in the literature have been in children or young adults. We report a case of eruptive collagenomas in later life. To our knowledge, no therapeutic interventions have been described.
A 32-year-old woman with a 3-year history of an intermittent painful and pruritic rash involving the lower extremities presented for evaluation. Outside biopsy obtained 30 months before presentation demonstrated superficial and deep perivascular dermal neutrophilic infiltrates, karyorrhexis, and fibrinoid mural necrosis consistent with leukocytoclastic vasculitis. Physical examination of the lower extremities revealed symmetrical firm pink-red papules and nodules coalescing into larger geographic plaques situated most prominently over the extensor knees and lateral ankles. Prominent petechia were also noted on the dorsum of both feet. Skin biopsies from 3 representative lesions revealed a narrow Grenz zone and marked perivascular and interstitial neutrophilic inflammatory infiltrate associated with leukocytoclasis and prominent fibrosis throughout the reticular dermis and subcutis. No microorganisms were identified with the PAS, Fite acid fast, and Gram stains. Findings were consistent with erythema elevatum diutinum (EED). While negative for markers of connective tissue disease and infectious agents, laboratory examination revealed markedly elevated IgA levels. Notably, the patient became pregnant ;1 month after presentation in our clinic, and suffered concomitant worsening of her disease. EED is an uncommon fibrosing cutaneous small vessel vasculitis with fewer than 200 cases reported to date. It is seen most commonly in patients aged 30 to 60 years, and typically presents as red-brown papules or nodules favoring the extensor extremities. EED has been associated with infections, autoimmune conditions, and hematologic disorders (IgA gammopathy most notably). EED is characterized by a chronic waxing and waning course that typically resolves within 5 to 10 years. Dapsone is the treatment of choice, and is particularly effective in early, nonfibrosed lesions. Alternative treatments with variable efficacy include tetracycline and niacinamide, NSAIDs, intralesional corticosteroids, colchicine, and plasmapheresis. In the present case, low-dose dapsone (25 mg daily) has been used under the guidance of the patient’s perinatologist for the past 6 months. The patient has experienced decreased inflammation but no change in lesion size on this regimen. Augmentation of therapy will be considered postparturition as merited by the clinical course.
Commercial support: None identified.
Commercial support: None identified.
P7091 Eruptive collagenomas of the skin in an adult woman Carmen Martinez Peinado, Dermatology Department, Almeria, Spain; Ana Menendez, Dermatology Department, Almeria, Spain; Francisca Simon, Dermatology Department, Almeria, Spain; M. Jose Alonso, Dermatology Department, Almeria, Spain; Mercedes Alcalde, Dermatology Department, Almeria, Spain; Pilar Gomez, Dermatology Department, Almeria, Spain Background: Eruptive collagenomas form part of a spectrum of connective tissue nevi. The major element of the extracellular connective tissue within a connective tissue nevus may be collagen, elastin fibers, or glycosaminoglycans. In a collagenoma, collagen predominates. Collagenomas can be classified into 4 groups and may be localized, generalized, acquired or inherited, isolated or in association with systemic malformations.
AB50
J AM ACAD DERMATOL
APRIL 2013
P7029
Epidermodysplasia verruciformis associated with HPV 10 Amir Zahir, MD, George Washington University Medical Faculty Associates, Washington, DC, United States; Alison Ehrlich, MD, MHS, George Washington University Medical Faculty Associates, Washington, DC, United States; Lauren Craig, George Washington University School of Medicine and Health Sciences, Washington, DC, United States; Peter Rady, MD, PhD, University of Texas Medical School at Houston, Houston, TX, United States; Stephen Tyring, MD, PhD, University of Texas Medical School at Houston, Houston, TX, United States Case report: A 42-year-old white man presented to our clinic with a 30-year history of reddish-brown papules on the extensor surfaces of his arms and anterior legs. He was treated with liquid nitrogen 9 years ago, and the treatment resulted in central clearing of his lesions. On examination, he had erythematous papules and macules on the extensor surfaces of his forearms. No clinical sign of cutaneous carcinoma was present. He has no personal history of skin cancer or immunosuppression. The patient was adopted and does not know if he has a family history of recalcitrant warts or skin cancer. A biopsy of a papule on his left arm revealed epidermal acanthosis, hyperkeratosis, vacuolation of superficial keratinocytes in the granular layer, and mild perivascular infiltrate in the superficial dermis. These findings are consistent with verruca plana. In situ hybridization was performed to detect multiple HPV subtypes. The tissues were analyzed for HPV types 2, 5, 6, 8, 11, 16, 18, 30, 31, 33, 35, 42, 43, 44, 45, 51, 52, 68, and 70. The results were all negative. HPV typing by nested PCR for the detection of epidermodysplasia verruciformis associated HPV types using an EV-HPV primer system was negative. Single PCR using the GP5+/6+ HPV primer system revealed a putative HPV fragment. The sequencing of the isolated PCR products and the computer assisted analysis (NCBI-BLAST) of the PCR products was identified as HPV type 10 DNA. Discussion: Epidermodysplasia verruciformis (EV) is a rare, inherited dermatologic condition demonstrating an increased susceptibility to specific HPV genotypes, resulting in both benign and malignant skin lesions. Acquired EV has been described in patients with HIV or in those who are on immunosuppressive therapy. EV lesions are frequently described as pityriasis versicolorelike scaly macules, flat wart-like papules, or as verrucous and seborrhic keratosiselike papules and plaques. These lesions range in pigmentation and often amalgamate to form patches and plaques. The classic histologic finding of epidermodysplasia verruciformis is a verruca planaetype lesion with mild to moderate hyperkeratosis, acanthosis, and enlarged vacuolated keratinocytes with perinuclear halos and blue-gray cytoplasmic pallor. We discuss a patient with EV who presents with plane wartetype skin lesions associated with HPV 10, a less frequently cited causative subtype, and histologic findings that are not classic for EV.
Erythema elevatum diutinum Fabienne Robuschi-Lestouquet, MD, Complejo Hospitalario Toledo, Toledo, Spain; Blas Gomez-Dorado, MD, Complejo Hospitalario Toledo, Toledo, Spain; Cristina Cardona-Alzate, MD, Complejo Hospitalario Toledo, Toledo, Spain; Cristina Schoendorff-Ortega, MD, Complejo Hospitalario Toledo, Toledo, Spain; Elena Vera, MD, Complejo Hospitalario Toledo, Toledo, Spain; Raquel Martin, MD, Complejo Hospitalario Toledo, Toledo, Spain Erythema elevatum diutinum (EED) is considered a chronic cutaneous vasculitis We reported 2 cases. Case 1 is a 59-year-old woman who presented with a 1-year history of itchy nodules on the elbows, and since the last week bilateral asymptomatic lower limb lesions. Her medical history was remarkable for rheumatic arthritis. Physical examination revealed erythematous to skin colored papules and nodules on her both elbows and forearm. Moreover, she had bilateral reddish to purple macules and papules on her legs. Laboratory investigations revealed anemia (Hb 10, 1 g/dL, hto 32%), protein C reactive, and VSG elevated. Skin biopsy was performed of the nodule from the elbow and histopathology revealed dermal fibrosis, dense dermal infiltrate composed of lymphocytes, and neutrophils. Leukocytoclastic vasculitis (LCV) with areas of fibrinoid necrosis and neutrophils infiltrating the vessels walls was seen. A diagnosis of EED was made and the patient is without treatment. Case 2 is a 50-year-old man who presented with year and half history of papules on the extensor surfaces of extremities and occasionally arthalgia. A physical examination revealed firm erythematous to skin-colored papules on his dorsal hands, elbows, knees, ankles and on left ear. Laboratory investigations revealed anemia (Hb 10, g/dL, hto 30%), VSG elevated. Histopathologic examinations of the skin lesions from the left forearm revealed vasculitis in the small vessels of the dermis with fibrinoid deposits and extravasated red blood cells. A diagnosis of EED was made and he was treated with dapsone 100 mg daily with a good response in 4 months. EED is considered to be a chronic fibrosing leukocytoclastic vasculitis. The cause is unknown, and it is believed to be an immune complexemediated vasculitis. EED has been linked to autoimmune processes, hematologic abnormalities, and infectious disease. Autoinmune processes include rheumatoid arthritis. Clinically typical lesions are erythematous, yellow-brown, papules and nodules.The extensor surfaces of the extremities are most commonly involved. Histopathologically acute lesions show a neutrophilic pervascular infiltrate with dermal fibrin deposits and leucocytoclasis. Mature lesions show dermal fibrosis and vasculature prominence with persistent inflammation with eosinophils, histiocytes, and lymphocytes. Treatment of EED is difficult because this disorder follows a chronic and recurrent course. Dapsone is considered first-line treatment for EED. Commercial support: None identified.
Commercial support: None identified.
P6067 Erythema elevatum diutinum in a 32-year-old pregnant woman Jason Michaels, MD, MS, Mayo Clinic, Scottsdale, AZ, United States; Catherine Harmon, MD, Mayo Clinic, Scottsdale, AZ, United States; David DiCaudo, MD, Mayo Clinic, Scottsdale, AZ, United States; James Yiannias, MD, Mayo Clinic, Scottsdale, AZ, United States
Case report: We present a case of eruptive collagenomas of the skin in a 68-year-old woman. No familal association was detected or systemic disorders associated. We describe the typical clinical features and histology of this rare condition. The differential diagnosis and other types of cutaneous collagenomas are briefly discussed. A biopsy was required to confirm the excessive mature collagen that predominates in these lesions. Discussion: Connective tissue nevi are circumscribed alterations in the development of the dermis that may be congenital or become apparent in later life. They are slowly progressive and permanent. Eruptive collagenomas can be distinguished from other collagenomas by history and examination. The incidence of eruptive collagenoma is not known but all cases described in the literature have been in children or young adults. We report a case of eruptive collagenomas in later life. To our knowledge, no therapeutic interventions have been described.
A 32-year-old woman with a 3-year history of an intermittent painful and pruritic rash involving the lower extremities presented for evaluation. Outside biopsy obtained 30 months before presentation demonstrated superficial and deep perivascular dermal neutrophilic infiltrates, karyorrhexis, and fibrinoid mural necrosis consistent with leukocytoclastic vasculitis. Physical examination of the lower extremities revealed symmetrical firm pink-red papules and nodules coalescing into larger geographic plaques situated most prominently over the extensor knees and lateral ankles. Prominent petechia were also noted on the dorsum of both feet. Skin biopsies from 3 representative lesions revealed a narrow Grenz zone and marked perivascular and interstitial neutrophilic inflammatory infiltrate associated with leukocytoclasis and prominent fibrosis throughout the reticular dermis and subcutis. No microorganisms were identified with the PAS, Fite acid fast, and Gram stains. Findings were consistent with erythema elevatum diutinum (EED). While negative for markers of connective tissue disease and infectious agents, laboratory examination revealed markedly elevated IgA levels. Notably, the patient became pregnant ;1 month after presentation in our clinic, and suffered concomitant worsening of her disease. EED is an uncommon fibrosing cutaneous small vessel vasculitis with fewer than 200 cases reported to date. It is seen most commonly in patients aged 30 to 60 years, and typically presents as red-brown papules or nodules favoring the extensor extremities. EED has been associated with infections, autoimmune conditions, and hematologic disorders (IgA gammopathy most notably). EED is characterized by a chronic waxing and waning course that typically resolves within 5 to 10 years. Dapsone is the treatment of choice, and is particularly effective in early, nonfibrosed lesions. Alternative treatments with variable efficacy include tetracycline and niacinamide, NSAIDs, intralesional corticosteroids, colchicine, and plasmapheresis. In the present case, low-dose dapsone (25 mg daily) has been used under the guidance of the patient’s perinatologist for the past 6 months. The patient has experienced decreased inflammation but no change in lesion size on this regimen. Augmentation of therapy will be considered postparturition as merited by the clinical course.
Commercial support: None identified.
Commercial support: None identified.
P7091 Eruptive collagenomas of the skin in an adult woman Carmen Martinez Peinado, Dermatology Department, Almeria, Spain; Ana Menendez, Dermatology Department, Almeria, Spain; Francisca Simon, Dermatology Department, Almeria, Spain; M. Jose Alonso, Dermatology Department, Almeria, Spain; Mercedes Alcalde, Dermatology Department, Almeria, Spain; Pilar Gomez, Dermatology Department, Almeria, Spain Background: Eruptive collagenomas form part of a spectrum of connective tissue nevi. The major element of the extracellular connective tissue within a connective tissue nevus may be collagen, elastin fibers, or glycosaminoglycans. In a collagenoma, collagen predominates. Collagenomas can be classified into 4 groups and may be localized, generalized, acquired or inherited, isolated or in association with systemic malformations.
AB50
J AM ACAD DERMATOL
APRIL 2013