Familial vulvar dystrophy of lichen sclerosus type

Abstract

Lichen sclerosus et atrophicus (LS&A) of the vulva and perianal skin is an atrophic condition that con occur alone or in association with additional lesions situated elsewhere on the skin surface. The cases of three sisters with LS&A of the vulva are reported herein; in one a squamous cell carcinoma developed in a hyperplastic area of mixed dystrophy (LS&A with foci of hyperplasia). Familial occurrences of LS&A are rare. Malignant neoplasms arising in areas of LS&A have been reported previously. Squamous cell carcinoma arises in hyperplastic areas of mixed dystrophy where there is evidence of both LS&A and epithelial hyperplasia.

Genital Vulvar Lichen Sclerosus in Monozygotic Twin Women: A Case Report and Review of the Literature

“…Both sexes are affected, both before and after puberty, with female predominance at all ages. Postmenopausal women and prepubertal girls are most frequently affected [1, 2]. The prevalence is about 1.7% in the general female population.…”