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PubMed · 7163741

The nose in anhidrotic ectodermal dysplasia.

L M Gil-Carcedo
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Abstract

A case is presented of anhidrotic ectodermal dysplasia, of extreme rarity. This is distinguished by disorders in the structures of ectodermal origin. The clinical characteristics--hypohidrosis, hypodontia, hypotrichosis-, and the genetic features--genealogy, karyotype--are examined first. The patient's face appears to be small, due to the combination of frontal bossing, hypodontia, and a depressed nasal dorsum, somewhat resembling the saddle nose of congenital syphilis. The case is studied from various perspectives: clinical, radiological, anatomicopathological and ultrastructural. The study concentrates principally on the anomalies of the nasal cavities, where there is a notable atrophy of the nasal mucosa. This atrophic rhinitis displays all the features of ozaena: severe crusting, fetid green secretion, nasal obstruction, etc.

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AnodontiaChild, PreschoolEctodermal DysplasiaHumansHypohidrosisMaleNasal CavityNasal MucosaCase ReportsJournal Article

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L M Gil-Carcedo. 1982. The nose in anhidrotic ectodermal dysplasia.. https://pubmed.ncbi.nlm.nih.gov/7163741/

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