Background Epidermodysplasia verruciformis (EV) is a rare, inherited d
isorder in which there is widespread and persistent infection by multi
ple subtypes of human papilloma virus, tinea versicolor-like lesions a
nd plaques, and frequently malignant manifestations. Materials and met
hods We report two cases of EV-a sister and brother aged 14 and 18 yea
rs respectively. Both had classical skin lesions together with neurolo
gical manifestations and deafness. In addition the man had plantar hyp
erkeratosis. They were treated with etretinate. Conclusions PCR and DN
A hybridization of skin lesions from the man contained HPV-20 and HPV-
57. He was treated with long-term oral acitretin; the warty lesions be
came 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.