[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.
Hautklinik und Poliklinik, Rheinischen Friedrich-Wilhelms-Universität Bonn.
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D000328:Adult; D000505:Alopecia / Q000235:genetics*; D003876:Dermatitis, Atopic / Q000235:genetics; D004535:Ehlers-Danlos Syndrome / Q000235:genetics*; D005260:Female; D006801:Humans; D007039:Hypotrichosis / Q000235:genetics*; D010375:Pedigree
Hypotrichosis congenita hereditaria Marie Unna mit Ehlers-Danlos-Syndrom und Atopie.