title: The wide spectrum of clinical expression in Adams-Oliver syndrome: a report of two cases.
abstract: Two children are described with the combination of aplasia cutis congenita (ACC) and transverse limb defects known as Adams-Oliver syndrome. Whereas in the first child the typical features of ACC, syndactyly and transverse nail dystrophy were only mildly expressed and associated defects of the central nervous system and cardiac malformations were absent, the second child suffered from a very severe expression of the syndrome, with a combination of ACC, syndactyly, cutis marmorata telangiectatica congenita and multiple cardiac and central nervous system malformations which resulted in fatal central respiratory insufficiency.
authors.affiliation: Klinik und Poliklinik für Dermatologie und Allergologie am Biederstein, Technische Universität München, Biedersteiner Strasse 29, 80802 Munich, Germany.
authors.forename: M
authors.initials: M
authors.lastname: Mempel
authors.forename: D
authors.initials: D
authors.lastname: Abeck
authors.forename: I
authors.initials: I
authors.lastname: Lange
authors.forename: K
authors.initials: K
authors.lastname: Strom
authors.forename: A
authors.initials: A
authors.lastname: Caliebe
authors.forename: A
authors.initials: A
authors.lastname: Beham
authors.forename: M
authors.initials: M
authors.lastname: Kautza
authors.forename: W I
authors.initials: WI
authors.lastname: Worret
authors.forename: B A
authors.initials: BA
authors.lastname: Neubauer
authors.forename: J
authors.initials: J
authors.lastname: Ring
authors.forename: H
authors.initials: H
authors.lastname: Schröder
authors.forename: R
authors.initials: R
authors.lastname: Fölster-Holst
mesh_terms: D000015:Abnormalities, Multiple; D001921:Brain / Q000002:abnormalities*; D004476:Ectodermal Dysplasia / Q000150:complications*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009264:Nails, Malformed; D012536:Scalp Dermatoses / Q000150:complications; D013576:Syndactyly / Q000150:complications*; D013577:Syndrome; D014034:Toes / Q000002:abnormalities*