Aggressive Squamous Cell Carcinoma Developed on Familial Epidermodysplasia Verruciformis Basis: Three Cases in a Family

摘要

Epidermodyplasia Verruciformis is a rare genetic disease, which is usually inherited in an automosal recessive manner. Flat, papular and macular skin lesions generally occur at the dorsal side of the hands, extremities and face at childhood due to defect of cell mediated immunity. These macular lesions progress to intraepithelial lesions and skin malignancies such as squamous cell carcinoma. It can also be seen in immune deficiency. It may be related to the sporadic gene mutations. Suspicious premalignant and malignant lesions should be resected with negative surgical margin. Patients and their relatives with suspicious lesions should be protected from sun exposure and followed up carefully. Intraepithelial neoplasia should be treated with CO2 laser therapy, photodynamic therapy or imiguimod. Three members of a family (two daughters and a son) with squamous cell carcinoma developed on epidermodisplasia verruciformis basis and treatment modalities are reported here.