Hay-Wells syndrome: a case report

Hay-Wells syndrome is a rare form of ectodermal dysplasia initially described by Hay and Wells in 1976. It is an autosomal dominant disorder with varying forms of expression featuring congenital abnormalities of the skin, hair, teeth, nails and sweat glands. The present report describes the case of...

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Bibliographic Details
Published in:Anais brasileiros de dermatología 2010-03, Vol.85 (2), p.232-235
Main Authors: Rosa, Dário Júnior de Freitas, Machado, Ronaldo Figueiredo, Martins Neto, Marcelino Pereira, Sá, Alessandra Almeida Montenegro de, Gamonal, Aloísio
Format: Article
Language:English
Subjects:
Abnormalities, Multiple
Adolescent
Cleft Palate
Ectodermal Dysplasia - diagnosis
Face - abnormalities
Humans
Male
Nails, Malformed
Phenotype
Syndrome
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Summary:Hay-Wells syndrome is a rare form of ectodermal dysplasia initially described by Hay and Wells in 1976. It is an autosomal dominant disorder with varying forms of expression featuring congenital abnormalities of the skin, hair, teeth, nails and sweat glands. The present report describes the case of a 17-year old white boy, the son of nonconsanguineous parents, who presented ankyloblepharon filiforme adnatum, ectodermal dysplasia and a cleft palate at birth, which are considered cardinal signs of this syndrome by most authors. We also highlight the importance of implementing multidisciplinary follow-up of these patients.
ISSN:1806-4841