Clinical aspects of epidermodysplasia verruciformis and review of the literature

Abstract

Background: Epidermodysplasia verruciformis (EV), is an unusual genodermatosis characterized by persistent human papilloma virus infection with an autosomal recessive inheritance pattern. Clinically, it is characterized by flat wart-like lesions, scaly hypo- and hyperpigmented macules and/or patches, which resemble pityriasis versicolor, and development of early beginning nonmelanoma cutaneous carcinomas.

Methods: The epidemiological and clinical features of seven cases with EV that have been followed up for 5 years were included in the study.

Results: Seven cases consisted of four males and three females. All seven cases were working outdoors. Three cases were the product of consanguineous marriages. The onset of the lesions was between the ages of 1-20 years (the average age was 9.29 years). The initial appearance of cutaneous tumors were between the ages 15-34 years (average age: 21.28 years). Six of seven cases had malignant cutaneous tumors, of which histopathological examination revealed squamous cell carcinoma (SCC). Three of our cases had radiotherapy previously for the existing SCCs. These cases had more early malignant transformations.

Conclusion: Radiotherapy applied against the SCC previously, together with sunlight exposure may cause early malignant transformation of skin lesions and the destructive tumors.