Abstract

Epidermodysplasia verruciformis (EV) is an autosomal recessive genodermatosis that predisposes the patient to skin lesions showing atypia of the epidermis. The lesions are the result of a complex interaction between an altered immune system and infection with the human papillomavirus (HPV). Three types of EV have been proposed: classical genetic, non-classical genetic, and acquired EV. The latter is usually observed in immunocompromised/immunosuppressed patients. However, differentiation between subtypes can be difficult.

A 65-year-old man came to the clinic with a recurrent lesion of the upper right eyelid with an unclear history of previous surgical treatment. The lesion occupied 50% of the upper-outer eyelid and consisted of yellow, crusted, boselated, focally horn-like epidermal tubercles, and measured 1.5 x 2 cm. A series of two biopsies revealed verruciform hyperplasia of the epidermis with mild-to-moderate cytologic atypia of keratinocytes. Most of the specimen showed a dysplastic epidermis, which contrasted sharply with the uninvolved and thinner epidermis in the background. In addition to dysplasia, hypergranulosis, hyperkeratosis, purplish-blue cytoplasm, enlarged keratohyalin granules, perinuclear halos and nuclear changes in some keratinocytes were observed, indicating the cytopathic effect of HPV. In Situ hybridization for HPV types 6, 11, 16, 18, 31, 33, and 51 results were negative (Mayo Clinic, Rochester, MN).

Epidermodysplasia verruciformis may increase the risk of malignant transformation into squamous cell carcinoma. The risk depends on the viral type and increases if the lesion is left untreated, especially in sun-exposed areas. The risk of carcinoma reaches 50% if HPV-5 and HPV-8