Surgery and etretinate in the therapy of epidermodysplasia verruciformis [CHIRURGIA ED ETRETINATO NEL TRATTAMENTO DELL'EPIDERMODISPLASIA VERRUCIFORME]

Epidermodyplasia verruciformis is a rare autosomal recessive genodermatosis characterized by disseminated chronic HPV 5,17,20 infection and by the onset of basal and prickle cell carcinomas and Bowen's disease in areas exposed to the light. Clinical case. A 49-year-old woman suffering from epidermodysplasia verruciformis and presenting numerous epitheliomas in sites exposed to light was submitted to a therapy with etretinate for 4 months. During the first month of therapy she underwent a plastic surgical treatment of the largest sized neoplasms. At the end of the pharmacological treatment, that was well tolerated by the patient, we observed a partial regression of the cancerous and precancerous lesions and a valid improvement in the skin condition. Discussion. Surgical therapy, though necessary for large-sized tumours, does not prevent the onset of new tumours and is irksome and mutilating for the patient. Etretinate proves useful in the therapy of epidermodysplasia verruciformis because it exerts an effect on the replication and differentiation of normal and neoplastic cells, stimulates the cell-mediated immune response and has a direct antiviral action. Conclusions. In the case we observed we subjected the patient to a combined pharmacological and surgical therapy. We find it difficult to distinguish between the effects of the two forms of treatment, but one year of follow-up demonstrates that a combined therapy of EV, surgical and pharmacological, is lowly mutilating for the patient but is useful in controlling the clinical picture of the disease.