Epidermodysplasia Verruciformis-A Genetic Disorder

Abstract

S. Swati*, K. Sowjanya, R. Lakuma, S. A. Sunaina, G. Srividya, V. Rohitha

Epidermodysplasia verruciformis also known as Tree man disease. It is recognized as an inherited disorder with wide spread and persistent with human papilloma virus causes defect in cell mediated immunity. It is a rare, lifelong, cutaneous, autosomal recessive genetic disorder of the immune system manifested by (HPV) infection beginning in the early years of life. This disorder was first described by Lewandowsky and Lutz 1 in 1922 as an epidermal nevus. In 1939, Sullivan and Ellis2 described a close relationship between EV and high risk of skin cancer. In EV, there are mutations in the EVER1 or EVER2 genes on chromosome 17q25, which, due to a defect of cell-mediated immunity, lead to an abnormal susceptibility of the patients to a specific group of HPV genotypes known as EV HPV. Most of these are HPV 5 and 8; more rarely, HPV 14,17, 20 and 47, and all have oncogenic po-tential3 In the addition, there is a link with squamous cell carcinoma. A tree man syndrome is a rare disorder happening more frequently at a younger age in the general population. It is an unusual Geno dermatosis character-ized by an autosomal recessive inheritance pattern. Non-surgical therapies and surgical therapies are known to treat this disorder, but it is known to be lifelong disorder. Recent surgery was performed in Bangladesh which is known to be successful