Ocular and cutaneous squamous cell carcinoma in an African American man with epidermodysplasia verruciformis resulting in blindness and death.

Abstract

Epidermodysplasia verruciformis (EV) is a hereditary disease that has served as a model for viral-induced carcinogenesis. Patients with EV have an increased susceptibility to infection with human papillomavirus, which results in the development of benign lesions in childhood. The lesions may resemble tinea versicolor in appearance and distribution, and usually remain benign. However, later in life cutaneous squamous cell carcinoma may develop on sun-exposed areas, such as the forehead. Radiation therapy may not only result in the malignant conversion of EV lesions, but may also increase the risk of metastases. To our knowledge, this is the first report of a patient with EV in whom squamous cell carcinoma of the conjunctiva developed. His clinical course was complicated by ocular invasion causing blindness, metastasis, and eventually death.