[Hypotrichosis congenita hereditaria Marie Unna with Ehlers-Danlos syndrome and atopy].

A family suffering from hypotrichosis congenita hereditaria Marie Unna is reported (16 members affected in five generations). The typical symptoms of this autosomal dominant syndrome were found: pili torti, canaliculi et trianguli, inborn generalized hypotrichosis, later on resulting in alopecia of the androgenetic type. A 19-year-old female patient with hyperandrogenemia was treated with cyproterone acetate and ethinyl estradiol. Furthermore, hypotrichosis was associated with Ehlers-Danlos syndrome and atopic symptoms in the last three generations of the family. There does not seem to be any genetic connection between the syndromes however.