[Syndromes 17. Hypohidrotic ectodermal dysplasia].
The X-linked hypohidrotic ectodermal dysplasia (HED) is characterized by hypohidrosis, hypotrichosis and hypodontia. Sebaceous and salivary glands ar also affected. Recognition of the syndrome usually comes from affected man, although female carriers may show symptoms. Dental treatment is complicated and should be coordinated by a dentist in a centre for special dental care. Cooperation with an orthodontist and an oral and maxillofacial surgeon is advocated.
Baart
J A
JA
Afdeling Mondziekten en Kaakchirurgie/Orale Pathologie, Academisch Centrum Tandheelkunde Amsterdam, (ACTA).
van Hagen
J M
JM
D000848:Anodontia / Q000235:genetics / Q000628:therapy; D041321:Chromosomes, Human, X; D004476:Ectodermal Dysplasia / Q000235:genetics* / Q000628:therapy; D005260:Female; D005808:Genes, Recessive; D008040:Genetic Linkage; D006801:Humans; D007007:Hypohidrosis / Q000235:genetics / Q000628:therapy; D007039:Hypotrichosis / Q000235:genetics / Q000628:therapy; D008297:Male