Abstract
Lichen sclerosus is a chronic inflammatory mucocutaneous disorder predominately affecting prepubertal girls and postmenopausal women. Isolated lichen sclerosus affecting the oral mucosa is exceedingly rare, and only 13 patients with biopsy-proved isolated oral disease have been reported in the literature. We report on a 7-year-old Korean girl with a well-demarcated 1.2x1.2 cm atrophic white plaque with an erythematous border and focal telangiectasia on the left vermillion lip, extending to the labial mucosa. No other cutaneous surfaces, including genitalia, were involved. An incisional biopsy of the plaque on the lip revealed a patchy lichenoid infiltrate of lymphocytes associated with sclerosis of the papillary dermis and a thinned epidermis consistent with a diagnosis of linear orofacial lichen sclerosus. Treatment with a short course of 1% pimecrolimus cream effectively prevented the progression of this lesion.