Epidermodisplasia verruciforme / Epidermodysplasia verruciformis

La epidermodisplasia verruciforme (EV) es una genodermatosis rara, caracterizada por el desarrollo de verrugas planas y lesiones similares a pitiriasis versicolor, asociada a infección crónica por Virus Papiloma Humano. Se reporta el caso de EV. Mujer de 49 años con múltiples lesiones verrucosas de larga evolución distribuidas en las cuatro extremidades y antecedente familiar de cuadro similar. Biopsia a los 19 años evidenció verrugas sin atipia. Nuevo estudio histológico informó carcinoma escamoso, decidiéndose extirpación de lesiones. La clínica inicia en edades tempranas, con elevado riesgo de transformación maligna. No existe tratamiento específico. Es esencial la fotoprotección estricta y manejo precoz de lesiones para prevención de progresión a carcinoma.

Epidermodysplasia verruciformis (EV) is a rare genodermatosis, characterized by the development of plane warts and pityriasis versicolor-like lesions, associated with chronic infection caused by the Human Papilloma virus. An EV case is reported. 49 year-old woman with multiple long standing warty lesions distributed on the four limbs and family background of similar cases. Biopsy at 19 years old evidenced warts without atypia. New histological study reported squamous cell carcinoma, deciding the removal of the lesions. The clinic begins at early age, with high risk of malignant transformation. There is no specific treatment. Strict photoprotection and early management of the lesions is essential to prevent the progression to carcinoma.

Acquired epidermodysplasia verruciformis in a renal transplant recipient - Case report

A 24-year-old male patient, who underwent kidney transplant six years ago due to Lupus nephritis, for the last two years presented asymptomatic erythematous scaly plaques on the abdomen and areas exposed to light. Post-transplantation immunosuppressive medications included prednisone, mycophenolate sodium and sirolimus. The histopathologic features were typical for epidermodysplasia verruciformis. Epidermodysplasia verruciformis is a rare autosomal recessive genodermatosis with increased susceptibility to specific strains of cutaneous human papilloma virus. The term ''acquired epidermodysplasia verruciformis'' was recently introduced to the literature and describes epidermodysplasia verruciformis occurring in patients with impaired cell-mediated immunity. We report an additional case associated to immunosuppression after kidney transplantation.

Epidermodysplasia verruciformis in a young man with HIV since birth - case report / Epidermodisplasia verruciforme em um jovem com HIV desde o nascimento - relato de caso

Epidermodysplasia Verruciformis is a genodermatosis characterized by susceptibility to infection by specific HPV types (HPV 3/10 and beta-HPVs). It is considered to be the first model in human carcinogenesis induced by HPV. In this report we present a rare case of Epidermodysplasia Verruciformis associated with vertical transmission of HIV. Although most patients with HIV present infections by HPV virus, the frequency of Epidermodysplasia Verruciformis is no greater in patients with HIV because in EV there is a deficiency of specific cellular immunity to infection by some types of HPV, called HPV-related Epidermodysplasia Verruciformis.

A Epidermodisplasia Verruciforme é uma genodermatose que se caracteriza pela susceptibilidade à infecção por tipos específicos de HPV (HPVs 3 e 10 e os beta-HPVs) e é considerada o primeiro modelo no homem de carcinogênese induzida pelo HPV. Neste artigo apresenta-se um caso raro de Epidermodisplasia Verruciforme em um paciente com HIV desde o nascimento. Apesar dos doentes com HIV apresentarem mais infecções pelos vírus HPVs, existem poucos relatos na literatura de Epidermodisplasia Verruciforme no doente com HIV. Isso se explica, pois a Epidermodisplasia Verruciforme é consequência de uma deficiência celular específica à infecção por alguns tipos distintos de HPVs, ditos HPVs relacionados a Epidermodisplasia Verruciforme.

Epidermodysplasia verruciformis: An unusual malignant transformation.

Epidermodysplasia verruciformis (EV) is a rare, life-long heritable disease caused due to a unique susceptibility to human papilloma virus. The disseminated verrucous lesions and pityriasis versicolor-like lesions persist from early childhood and can transform into a cutaneous malignancy in a fourth of patients. Malignant transformation into syringoid eccrine carcinoma (SEC) has been reported only once so far. SEC is an extremely invasive, rare, locally destructive, slowly growing adnexal tumor. We hereby report the association of EV with SEC in a 29-year-old male.

Epidermodisplasia verruciforme: apresentação clínica com variadas formas de lesões / Epidermodysplasia verruciformis: clinical presentation with varied forms of lesions

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 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.

Caso para diagnóstico / Case for diagnosis

Paciente do sexo feminino, parda, de 27 anos de idade, apresentando há três anos, placas hipocrômicas planas, ovaladas, de superfície áspera, no dorso e membros superiores com aspecto que lembrava pitiríase versicolor. Refere história familiar de um irmão cujas lesões são semelhantes. Exames laboratoriais, incluindo anti-hiv, sem alterações e com histopatológico evidenciando queratinócitos, aumentados de volume, com citoplasma basofílico e microvacuolado ocupando a porção superior da camada espinhosa e a granulosa. Após correlação anátomo-clínica, confirmou-se o diagnóstico de epidermodisplasia verruciforme pitiríase versicolor like.

A 27-year-old mixed-raced (pardo) female patient presented with flat ovate hypochromic plaques with a rough surface on the back and upper limbs, with an aspect resembling pityriasis versicolor. She reports family history involving a brother with similar lesions. Lab tests, including anti-HIV, showed no alterations and a histopathological examination showed enlarged keratinocytes with basophilic and microvacuolated cytoplasm occupying the upper portion of the spinous layer and the granulous layer. After anatomic-clinical correlation, the diagnosis of epidermodysplasia verruciformis pityriasis versicolor like was confirmed.

Epidermodysplasia verruciformis associated with isolated IgM deficiency.

A 20 year-old man presented to our clinic with multiple warts on both hands and tumoral lesions on his face but otherwise healthy. On dermatological examination, numerous brown-black papular lesions, changing from 2 to 5 cm in diameter were found on his face along with multiple, flesh-coloured, flat-topped papules on the dorsa of his hands. A tumoral lesion, approximately 3 cm in diameter on the right side of his forehead and desquamated erythematous macules were also observed on the trunk. Laboratory investigations showed that serum immunoglobulin M (IgM) level was decreased. The histopathological examination of verrucous lesions on the hands was consistent with epidermodysplasia verruciformis and the histopathological diagnosis of the tumoral lesion was squamous cell carcinoma.