title: [Congenital atrichia associated with keratin cysts - Variant of partial ectodermal dysplasia].
abstract: This is a report of three male siblings suffering from almost complete Atrichia, dental and osseous anomalies and also multiple disseminated horny papules. These latter lesions are caused by follicular plugging with a horny substance possibly due to a disorder of keratinisation as seen in patients with partial ectodermal dysplasia. Clinical manifestations and possible variations of this syndrome are described and the hereditary patterns are discussed.
mesh_terms: D000015:Abnormalities, Multiple / Q000473:pathology; D000505:Alopecia / Q000150:complications / Q000151:congenital* / Q000235:genetics / Q000473:pathology; D001842:Bone and Bones / Q000002:abnormalities; D002648:Child; D004476:Ectodermal Dysplasia / Q000150:complications* / Q000473:pathology; D006801:Humans; D008297:Male; D010375:Pedigree; D014071:Tooth Abnormalities / Q000150:complications
vernacular_title: Atrichia congenita mit Hornzysten - Variante einer partiellen ektodermalen Dysplasie.