'Typical' or 'classic' epidermodysplasia verruciformis (EV) is a rare genodermatosis characterized by increased susceptibility to infection with specific human papillomavirus (HPV) genotypes, usually beta-HPV strains, and to the oncogenic potential of some of them, mainly HPV5 and HPV8. Affected individuals develop disseminated flat wart-like and pityriasis versicolor-like cutaneous lesions in childhood or adolescence. The lesions are highly resistant to treatment. About half of patients with EV develop skin cancer, including cutaneous Bowen carcinomas in situ and invasive squamous cell carcinomas, mainly on sun-exposed areas. Individuals with typical or classic EV have no additional immunologic abnormalities (Ramoz et al., 2000; review by Beziat, 2020). [from
OMIM]