[Case of primary hypogonadism in congenital dyskeratosis: Zinsser-Cole-Engmann syndrome].

A rare case is described with congenital dermatosis, leading to systemic lesions of epithelium of skin and mucose, combined with primary hypogonadism. A very low level of testosterone was established with high gonadotropic hormones and prolactin. Hypogonadism was established to be the result from systemic changes of skin, its accessory and epithelium of seminiferous tubules in testes.