Epidermodysplasia verruciformis associated with peripheral T-cell lymphoma, squamous cell carcinoma, and langerhans cell histiocytosis

Abstract

Epidermodysplasia verruciformis (EV) is an extremely rare autosomal recessive genodermatosis characterized by abnormal susceptibility to human papilloma virus (HPV) infection. The inability to terminate HPV infection leads to widespread and persistent wart-like and macular skin lesions. The extent of cutaneous inivolvement and progress of the lesions vary considerably, but the persistence of skin lesions throughout the life is a characteristic feature of EV. The interaction among potentially oncogenic HPV genotypes, ultraviolet radiation, altered immunity, and genetic factors will lead to the development of nonmelanoma skin cancers in 30%–60% of cases. Although there is lifelong increased risk of squamous cell carcinoma, the association with hematological or solid malignancies is rare. Literature search shows very few isolated reports of non-Hodgkin's lymphoma, astrocytoma, thymoma, adenocarcinoma intestine, and leiomyosarcoma in patients with EV. Herein, we report a case of EV with an uncommon occurrence of peripheral T-cell lymphoma, squamous cell carcinoma, and Langerhans cell histiocytosis. EBV-associated squamous cell carcinoma carries increased number of viral proteins and this may play role in cell immortalization, chromosomal destabilization, and activation of telomerase. The development of lymphoma and Langerhans cell histiocytosis may be due to the defect in immune system and due to the oncogenic potential of the virus. These point to the importance of regular long-term follow-up of EV patients for the possible cutaneous and extracutaneous malignancy.