Extensive extra genital Csillag’s disease: a rare case report

Abstract

Lichen sclerosus et atrophicus (LSA), also called as Csillag’s disease, is characterized by small, shiny, porcelain white, sclerotic papules, plaques and atrophic patches that can occur at any site on the skin including the mucosa. Most commonly found in genitalia, rarely occurring on extragenital skin. Predominantly seen in women with bimodal age distribution. Predominantly an interface dermatoses histopathologically consisting of epidermal atrophy, sclerosis /homogenization of collagen fibers and lichenoid inflammatory infiltrates in dermis. No effective treatment till date although topical steroids, calcineurin inhibitors, topical retinoids and systemic agents like hydroxychloroquine, mexthotrexate, PUVA shows varying inconsistent results. Here we report a case of 36-year-old female having LSA with extensive extra vulval involvement including face. No any other co-existing skin disorders. She showed partial improvement use of physical sunscreens, systemic antimalarials, oral methotrexate and antioxidants.