Lichen sclerosus et atrophicus (LSA) may be found in males and females of all ages (1,2), but is typically a problem in older women appearing in the vulvar "keyhole" distribution (3,4). While it is generally assumed that LSA is an acquired disease, there are no longitudinal studies demonstrating its origin in normal skin, so that the possibility of congenital predisposition cannot be ruled out. Because the disease is occasionally found in premenarchal girls, an argument for congenital origin or genetic control may have some weight.
Attention has been paid to a potential defect in the immunological process leading to the development of LSA although laboratory evidence is not generally supportive (5-7). The mechanism leading to the histologic appearance of the dermis in LSA has not been elucidated but could be related to some deranged local tissue response, leading to the loss of defined architecture referred to as "homogenization" and the chronic inflammatory dermal response with round-cell infiltrate. A therapeutic inference from this consideration led to trials of topical tacrolimus and pimecrolimus that as macrolide immunosuppressants may be effective by inhibiting T-cell activation and cytokine production (6,8).
A familial tendency to LSA has been noted on occasion (9) but with only an occasional phenotypic demonstration, there is no significant impact on understanding this disease. I have personally seen a postmenopausal patient with LSA, whose sister also had the disease and I treated another patient with coexistent LSA and squamous cell carcinoma (SCC) whose daughter had severe LSA but no SCC. The issue of genetic predisposition would be of greater importance if a preventive therapy were available.
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