Epidermodysplasia Verruciformis

Excerpt

Epidermodysplasia verruciformis is a rare genetic dermatologic condition in which patients show a compromised immunologic ability to defend against and eradicate certain types of human papillomavirus (HPV). This leads to persistent infections and an increased lifetime risk of developing cutaneous dysplasia and malignancy. The connection between viral infections and certain forms of carcinogenesis, especially with HPV, is well established. Specific types of HPV are more strongly associated with the development of dysplasia and carcinogenesis.

Individuals with this condition develop HPV-derived cutaneous lesions at a significantly 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. In addition, this also leads to the development of Bowen disease and squamous cell carcinoma. The lesions associated with epidermodysplasia verruciform tend to exhibit a characteristic histopathologic appearance. Although epidermodysplasia verruciformis is a rare genetic disease, extensive research has provided insights into viral infection and its role in carcinogenesis pathways. This condition exists in broadly 2 forms:

1. The classic form is also the inherited or primary and follows an autosomal recessive pattern.

2. The acquired form is the secondary type and is clinically almost indistinguishable. This is mainly observed in HIV-infected, immunocompromised, or immunosuppressed individuals.