Epidermodysplasia verruciformis type 2

The disease is characterized by chronic infection with human papillomavirus (HPV). Widespread skin eruptions of flat-to-papillomatous, wartlike lesions and reddish brown pigmented plaques on the trunk, the hands, the upper and lower extremities, and the face are characteristic. The lesions may transform into malignant carcinomas, usually after age 30 years. Skin cancers initially appear on sun-exposed areas, such as the face and the ear lobes. Patients with EV are usually infected with multiple types of HPV. More than 30 HPV types, including types 3, 5a, 5b, 8-10, 12, 14, 15, 17, 19-21, 23-26, 37, 38, and 47, have been identified in EV tumors. EV results from an abnormal susceptibility to specific related human papillomavirus (HPV) genotypes and to the oncogenic potential of some of them, mainly HPV5. Infection with EV-associated HPV leads to the early development of disseminated flat wart-like and pityriasis versicolor-like lesions. Patients are unable to reject their lesions, and cutaneous Bowen carcinomas in situ and invasive squamous cell carcinomas develop in about half of them, mainly on sun-exposed areas. The lesions often resemble verrucae planae. The mucous membranes, hair, and nails are not affected. Malignant degeneration, usually of the superficial basal cell type, is frequent. Characteristic changes in the epidermal cells with peculiar vacuolization are observed.

Therapeutic options

  • Experimental therapies include intralesional administration of interferons and retinoids. These therapies have resulted in only a partial or transitory effect. In advanced HPV-related carcinomas, an experimental therapy involves treatment with a combination of 13-cis retinoic acid and interferon alpha or cholecalciferol analogues. For localized multiple malignant lesions, autotransplantation of skin from uninvolved skin has been reported with success in preventing further development of cancers. UV-B and UV-A exposure as well as x-ray irradiation should be avoided.
  • Epidermodysplasia verruciformis, eMedicine