Epidermodysplasia Verruciformis Associated with Astrocytoma, Mantle Lymphoma and Hepatitis B Virus Infection

Abstract

Epidermodysplasia verruciformis (EV) is a lifelong, rare autosomal recessive genetic hereditary skin disorder characterized by a unique susceptibility to human papilloma virus, associated with a high risk of malignant transformation. The disseminated verrucous lesions and pityriasis versicolor-like lesions persist from early childhood and can transform into a cutaneous malignancy in a fourth of patients. No definitive treatment against Epidermodysplasia verruciformis has been found yet. Extracutaneous cancers reported in Epidermodysplasia verruciformis are intestinal adenocarcinoma, plasmablastic lymphoma and leiomyosarcoma. Mantle cell lymphoma is uncommon under the age of 40 years and rare in patients under the age of 30 years. Here we are reporting a 19 year male patient with Epidermodysplasia verruciformis, astrocytoma and pulmonary tuberculosis who presented with symptoms of weight loss, indigestion and burning epigastric pain four months duration, Upper GIT endoscopy showed a gastric mass with active peptic ulcers. Biopsy revealed Mantle cell lymphoma. We investigated further and found to have immunodeficiency and hepatitis B virus infection. EV patients should receive regular follow-up for possible cutaneous or extracutaneous malignancy.