Familial lichen sclerosus et atrophicus in association with CREST syndrome: a case report.

Lichen sclerosus et atrophicus is an uncommon disease which appears to be multifactorial in aetiology. We describe a case of a young woman with CREST syndrome (calcinosis, Raynaud's phenomenon, oesophageal dysfunction, sclerodactyly and telangiectasia) who has a documented family history of two sisters with lichen sclerosus et atrophicus. She presented with vulvar pruritus in association with dyspareunia, and biopsy of atrophic white vulvar lesions was consistent with lichen sclerosus et atrophicus. Lichen sclerosus et atrophicus has been previously noted to occur in association with morphoea and lichen planus, although it has never been reported in conjunction with CREST syndrome.