Epidermodysplasia Verruciformis

Excerpt

The connection between viral infection and certain forms of carcinogenesis has been well established, especially with regards to human papillomavirus (HPV). Specific types of HPV have been implicated more strongly with carcinogenesis and the spectrum of dysplasia development. Epidermodysplasia verruciformis (EV) is a genetic dermatologic condition in which patients show a decreased immunologic ability to defend against and eradicate certain types of HPV, leading to persistent infection and increased lifetime risk of development of cutaneous dysplasia and malignancy. Persons with this disease develop HPV-derived cutaneous lesions at a much higher rate than the general population. This genodermatosis manifests mainly as verrucous cutaneous lesions such as multiple persistent verrucae, pityriasis versicolor-like lesions, and other verrucous or "warty" cutaneous lesions as well as the development of Bowen disease and squamous cell carcinoma. The lesions of EV tend to exhibit a characteristic histopathologic appearance. EV is a very rare genetic disease, but much research has been done on this entity as it has allowed insights into viral infection and their role in carcinogenesis pathways. There are broadly 2 forms of EV, the classic form being the inherited or primary type, inherited in an autosomal recessive pattern, while a separate acquired or secondary type is a clinically almost indistinguishable condition that is observed mainly in HIV-infected, immunocompromised, or immunosuppressed individuals.