Bazex-Dupre-Christol syndrome (BDCS) is an X-linked dominant disorder characterized by a triad of congenital hypotrichosis, follicular atrophoderma affecting the dorsa of the hands and feet, the face, and extensor surfaces of the elbows or knees, and the development of basal cell neoplasms, including basal cell nevi and basal cell carcinomas from the second decade onward (Yung and Newton-Bishop, 2005). Inoue et al. (1998) reviewed reports of a total of 125 patients with Bazex syndrome in 20 pedigrees. The disorder had been reported chiefly in France, and all of the patients were Caucasians. Hypotrichosis and follicular atrophoderma were the most commonly reported features, present in 84% and 83% of the patients, respectively; milia were reported in 76%, hypohidrosis in 54%, and nevoid basal cell carcinoma in 41%. Rombo syndrome (180730) has similar features, but shows autosomal dominant inheritance.