Epidermodisplasia verruciforme: apresentação clínica com variadas formas de lesões

Abstract

Epidermodysplasia verruciformis is a rare inherited skin disorder spread by HPV, with cases linked to chromosome X. It is characterized by hypo-or hyper-pigmented macular lesions, pityriasis versicolor-like lesions and an early tendency to develop skin malignancies. We present a case of epidermodysplasia verruciformis with a variety of lesions such as multiple plane warts, pityriasis versicolor-like lesions and aggressive squamous cell carcinoma on the face. Keywords: Carcinoma, squamous cell; Keratosis, actinic; Epidermodysplasia verruciformis; Warts Resumo: Epidermodisplasia verruciforme é uma genodermatose rara caracterizada por infecção disseminada por HPV, de caráter recessivo, com casos ligados ao cromossoma X. É caracterizada clinicamente por lesões maculares hipo ou hiperpigmentadas, lesões pitiríase versicolor like, verrugas planas e desenvolvimento precoce de carcinomas cutâneos. Descreve-se um caso de paciente com quadro clínico exuberante, apresentando todas as formas de lesões desta doença, inclusive presença de carcinoma espinocelular agressivo na face.

Epidermodysplasia Verruciformis: A Case Report

“…Epidermodysplasiaverruciformis, first described by Lewandowski and Lutz in 1922,is a rare, lifelong disorder of cell mediated immunity (CMI) with no sex, racial or geographic predisposition and is inherited in an autosomal recessive fashion. 1,4 The gene involved are EVER1 (Epidermodysplasia Verruciformis Enhancing Region) and EVER2. Mutation in these gene causes an individual to be susceptible to develop EDV.…”

“…E pidermodysplasiaverruciformis(EDV) is a rare disease characterized by susceptibility to infections caused by human papillomaviruses (HPV) specifically HPV 5, 8, 9, 12, 14, 15, 17, 19 -25 and due to defect in cell mediated immune response. 1 The skin lesions start in childhood and are predominantly seen in the sun exposed sites. 2 Lesions can present as widespread skin eruptions of flat to papillomatous wart-like lesions and reddish brown pigmented plaque on the trunk, extremities and face, and can also present as pityriasis versicolor-like lesions.…”

Acquired epidermodysplasia verruciformis in a renal transplant recipient - Case report

“…Among the therapeutic options are topical and systemic retinoids with antiproliferative action, imiquimod, cryotherapy, 5-fluorouracil and topical cidofovir, none of them with proven effectiveness. 1 Interferon can be utilized due to its antiviral action mechanism, besides stimulating T cells. With prolonged life expectancy of immunosuppressed patients it is fundamental that patients undergo complete dermatological exams periodically, to detect skin alterations resulting from cell immunity defects, among which EV stands out due to probable risk of carcinoma development.…”

“…Its onset is usually in early childhood, with the appearance of hypo or hyperpigmented macules, pityriasis versicolor-like lesions, flat warts and early development of skin carcinomas. 1,2 There are at least 20 types of HPVs associated with EV, which include types 3, 5, 8, 9, 10, 12, 14, 15, 17, 19-25, 38, 29, 36, 46, 47, 49 and 50. 3 These viruses have oncogenic potential in these patients and around 30 to 60% of affected patients may develop squamous cell carcinoma, usually in areas exposed to sunlight and in the fourth decade of life.…”

Epidermodysplasia Verruciformis acquired in an HIV-positive patient

“…The clinical picture is usually insidious, polymorphic, and maybe of the type flat warts, macular pityriasis Versicolor lesions, psoriasiform erythematous lesions, atrophic lenticular lesions, and may present a wide spectrum of shades 5,6 . With the most prolonged survival of immunosuppressed patients, a form of the disease called Epidermodisplasiaverruciforme acquired from clinical behavior similar to genetic disease has been described 2,3,7 . Most of the time, these patients will develop their lesions in adulthood.…”