Epidermodysplasia verruciformis: revision of a model of carcinogenic disease

This review addresses current issues regarding epidermodysplasia verruciformis (EV), which are relevant in clinical practice and to comprehend the mechanisms by which human papillomavirus (HPV) acts in cutaneous carcinogenesis. EV is an unusual genodermatosis, related to beta-HPV, with high risk for developing skin cancer. Clinical manifestations begin in childhood and they are characterized by flat warts, pityriasis versicolor-like macules and seborrheic keratoses lesions. Up to 50% of EV patients develop nonmelanoma skin cancer in sun exposed areas, mainly squamous cell carcinomas (SCC). Unlike genital carcinomas associated to alpha-HPV, in which transcriptionally active viral genomes invariably occurs, the EV-HPV seems to act by interaction with the host cell, the ultraviolet radiation and immunosuppression to result in carcinogenesis. The EV diagnosis is clinical and it can be confirmed by characteristic histopathological findings and EV-HPV identification. Until now, there is no effective treatment for EV. EV patients need regular clinical follow-up for early detection and treatment of cutaneous carcinomas. Despite the rarity of EV, the study of this genodermatosis is important to better understand the process of beta-HPV carcinogenesis.