Epidermodysplasia verruciformis with neurological manifestations

Epidermodysplasia verruciformis (EV) is a rare, inherited disorder in which there is widespread and persistent infection by multiple subtypes of human papilloma virus, tinea versicolor-like lesions and plaques, and frequently malignant manifestations.

We report two cases of EV-a sister and brother aged 14 and 18 years respectively. Both had classical skin lesions together with neurological manifestations and deafness. In addition the man had plantar hyperkeratosis. They were treated with etretinate.

PCR and DNA hybridization of skin lesions from the man contained HPV-20 and HPV-57. He was treated with long-term oral acitretin; the warty lesions became partly or wholly flattened and the plantar hyperkeratosis showed a remarkable improvement. The woman died 10 years later as a result of metastasizing breast cancer.