BACKGROUND: Epidermodysplasia verruciformis (EV) is a rare genodermatosis characterized by disseminated infection caused by specific types of human papillomavirus (HPV), development of cutaneous malignant tumors and immunological disturbances. OBJECTIVE: To correlate the clinical features of a group of 13 patients with EV aiming to contribute to knowledge of disease. METHOD: The clinical evaluation of 13 EV patients during 3 years. The clinical diagnosis was confirmed by histopathological and immunohistochemical findings. RESULTS: The EV began in childhood with flat warts and/or erithematous macules on the face and neck. The parental consanguinity was observed in most patients (12/13). Clinically the lesions were highly polymorphic with flat wart-like lesions, pityriasis versicolor-like lesions, erithematous macules and seborrheic keratoses-like lesions. The malignant conversion of lesions occured in 8 patients (62%). The tumor growth provoked heavy tissue loss in 50% of patients, and metastatic tumors caused death in 25%. CONCLUSION: The EV presents high familial incidence and an autosomal recessive mode of inheritance. The high polymorphism clinical doesn't affect the scalp and mucous membrane. The most frequent clinical manifestation is the "malignant" (62%), followed by "benignant" (23%) and "mixed"(15%). The cutaneous tumors are frequent, multiple, destructive, metastasize and cause death.
Epidermodysplasia verruciformis; papillomavirus; human
