ENT expression of hypohidrotic ectodermal dysplasia

Hypohidrotic Ectodermal Dysplasia (HED) is a rare recesive genetic disease linked to chromosome X whose main characteristic is the reduction of sweat glands, leading to a deficient sweating and an increase in body temperature. In HED mainly the ectodermal structures are involved such, as epidermis a...

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Published inActa otorrinolaringológica española Vol. 56; no. 4; p. 176
Main Authors González García, J, Galera Ruiz, H, Muñoz Borge, F
Format Journal Article
LanguageEnglish
Spanish
Published Spain 01.04.2005
Subjects
Adult
Ectodermal Dysplasia - complications
Ectodermal Dysplasia - diagnosis
Humans
Male
Otorhinolaryngologic Diseases - etiology
Tomography, X-Ray Computed
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Summary:Hypohidrotic Ectodermal Dysplasia (HED) is a rare recesive genetic disease linked to chromosome X whose main characteristic is the reduction of sweat glands, leading to a deficient sweating and an increase in body temperature. In HED mainly the ectodermal structures are involved such, as epidermis and its anexes (hair and nails), although non-ectodermal tissue may also become involved. Otolaryngologicalmanifestations are related to hypoplasia of the mucous glands of the upper aerodigestive tract, as chronic infections, like rhinitis, pharyngitis, bronchitis and otitis, and also epistaxis, dysphagia, anodontia and, ozena, among others. A case of a young adult male affected with HED who is referred to the Otolaryngology Departament with a history of chronic pharyngitis and ozena, is presented and the literature reviewed.
ISSN:0001-6519
DOI:10.1016/s0001-6519(05)78595-2