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  • Title: The nose in anhidrotic ectodermal dysplasia.
    Author: Gil-Carcedo LM.
    Journal: Rhinology; 1982 Dec; 20(4):231-5. PubMed ID: 7163741.
    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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