[Familial congenital hypotrichosis with "uncombable hair," retinal pigmentary dystrophy, juvenile cataract and brachymetacarpia: another entity of the ectodermal dysplasia group].

A new ectodermal dysplasia syndrome is reported in a family of Caucasian ancestry. The syndrome consists of congenital hypotrichosis with "uncombable hair", juvenile cataract, retinal pigmentary dystrophy, oligodontia and brachymetacarpia, and is inherited as an autosomal dominant trait.