Do you know this syndrome? Clouston syndrome

Ectodermal dysplasias are conditions that present primary defects in two or more tissues of ectodermal origin and can be classified as hypohidrotic and hidrotic. Hidrotic ectodermal dysplasia or Clouston syndrome is an autosomal dominant genodermatosis and appears as a triad of clinical findings: pa...

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Bibliographic Details
Published in:Anais brasileiros de dermatología 2017-06, Vol.92 (3), p.417-418
Main Authors: Sanches, Sarah, Rebellato, Priscila Regina Orso, Fabre, Andréa Buosi, Campos, Giovana Liz Marioto de
Format: Article
Language:English
Subjects:
Adolescent
DERMATOLOGY
Ectodermal dysplasia
Ectodermal Dysplasia - diagnosis
Female
Humans
Hypotrichosis
Keratoderma, Palmoplantar - diagnosis
Nail Diseases - diagnosis
Palmoplantar keratoderma
Syndrome
Syndrome in Question
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Summary:Ectodermal dysplasias are conditions that present primary defects in two or more tissues of ectodermal origin and can be classified as hypohidrotic and hidrotic. Hidrotic ectodermal dysplasia or Clouston syndrome is an autosomal dominant genodermatosis and appears as a triad of clinical findings: palmoplantar keratoderma, nail dystrophy, and hypotrichosis. The hair is sparse and brittle. The nails become thickened and dystrophic, which is an essential characteristic of the syndrome. The diagnosis is made based on clinical findings. This study reports a case of a patient who began with changes in hair, nails and palmoplantar keratoderma in early childhood.
ISSN:0365-0596
1806-4841
1806-4841
0365-0596
DOI:10.1590/abd1806-4841.20175716