Epidermodisplasia verruciforme: reporte de una serie de casos

La epidermodisplasia verruciforme es una genodermatosis rara que condiciona una susceptibilidad anormal a infecciones por el virus del papiloma humano, presentando lesiones cutaneas con potencial oncogenico. Objetivo: determinar la frecuencia de epidermodisplasia verruciforme y caracterizas los hallazgos demograficos, clinicos y terapeuticos de los pacientes que acudieron a la Catedra de Dermatologia del Hospital de Clinicas, entre los anos 2003, 2012. Material y metodos: se realizo un estudio descriptico de pacientes con diagnostico clinico e histopatologico de epidermodisplasia verruciforme. Resultados: se encontraron cinco casos. Todos los pacientes iniciaron el cuadro en la infancia y presentaron lesiones tipo pitiriasis versicolor y verrugas planas. Todos refirieron exposicion solar cronica y familiares de primer grado con la misma enfermedad. La neoplasia mas frecuente fue al enfermedad de Bowen, seguida del carcinoma epidermoide infiltrante. Los tratamientos incluyeron cirugia, crioterapia y 5-fluorouracilo topico...(AU) Epidermodysplasia verruciformis is a rare genodermatosis characterized by an abnormal susceptibility to human papiloma virus infection, developing cutaneous lesions with oncogenic potential. Objective: To determine the frequency, clinical demographic and therapeutic characteristics of patients who attend to the Dermatology Department of the Hospital de Clinicas between 2003 and 2012. Methods: A descriptive study of patients with clinical and histopathological diagnosis of epidermodysplasia verruciformis was carried out. Results: Five cases were found. Childhood-onset of disease, pityriasis versicolor-like lesions and flat warts were seen in all cases. All patients reported chronic sun exposure and first-degree relatives with the same disease. BowenAEs disease was the most frequent neoplasm, followed by invasive squamous cell carcinoma. Treatment modalities included surgery, cryotherapy and topical 5 fluorouracil. Discussion: Consistently to the infrequent nature of the disease, only five cases were diagnosed in the years. Unlike other studies, BowenAEs disease was the predominant neoplasm...(AU)