X-LINKED INHERITANCE OF EPIDERMODYSPLASIA VERRUCIFORMIS - GENETIC AND VIROLOGIC STUDIES OF A KINDRED

A family with typical epidermodysplasia verruciformis (EV) is described in which only male members were affected. None of the index patient''s 10 children had EV, but 4 of 8 grandsons born to his daughters inherited the disorder. All were infected with human papillomavirus (HPV) 3 and HPV 8. The inheritance in this kindred most likely results from an X-linked recessive genetic defect. Since other kindreds were described with autosomal inheritance, this novel inheritance pattern suggests that the persistent high clinical susceptibility to HPV infection characteristic of EV may result from defects in either of at least 2 different genetic loci, 1 of which may be located on the X chromosome.