01392nas a2200217 4500000000100000008004100001260001200042653002700054653002200081653001200103100001500115700001200130700001200142700001200154245013400166856007500300300001200375490000700387520076600394022001401160 2021 d c01/202110aCell mediated immunity10aepidermodysplasia10aleprosy1 aTripathy D1 aNeema S1 aKinra P1 aSinha P00aLepromatous leprosy in a case of epidermodysplasia verruciformis - A coincidence or a result of defective cell-mediated immunity? uhttps://www.ijmyco.org/temp/IntJMycobacteriol102210-3371611_092156.pdf a210-2130 v103 a

Epidermodysplasia verruciformis (EDV) is a rare, autosomal recessive, genetic disorder of immune dysregulation characterized by increased susceptibility to cutaneous human papilloma virus infections. It was first described by Lewandowsky and Lutz in 1922 as a form of epidermal nevus. Its association with skin cancers was proposed by Sullivan and Ellis in 1939. Initial lesions often occur in childhood and are of two types; lifelong eruptions of pityriasis versicolor like lesions and flat wart like papules. The latter is characterized by malignant transformation in 35%-50% of patients especially after 40-50 years of age. Bowen disease is the most common tumor followed by invasive squamous cell carcinoma, however, metastasis is rarely encountered.

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