Epidermodysplasia verruciformis: three case reports and a brief review

Abstract

Epidermodyplasia verruciformis (EV) is a rare heritable disease that is characterized by an increased susceptibility to infection with specific human papillomavirus (HPV) types due to a defect in cell-mediated immune response to HPV infection. Widespread infection with HPV is responsible for the development of wart-like lesions and pityriasis versicolor-like spots. These individuals have a lifelong risk of developing cutaneous malignancies, especially Bowen's disease and squamous cell carcinoma, mainly in sun-exposed parts. Being the first disease to correlate cancer and viral infection, EV serves as the cornerstone of the understanding of viral oncogenesis. We report three cases of EV, of which one patient subsequently developed Bowen's disease. The patients had multiple hypopigmented papules and plaques of varying sizes that started erupting in childhood and were mainly distributed over sun-exposed parts of the body. Histopathology of the skin biopsies was consistent with EV. One of these patients had started developing an ulcerated plaque over the left clavicle 2 years earlier. A biopsy from the clavicular region also showed histopathological features of Bowen's disease arising in EV. Hence, this clinical review discusses three cases of EV presenting in different age groups with detailed histopathological findings typical for EV.