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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...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Sociedade Brasileira de Dermatologia
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5514592/ https://www.ncbi.nlm.nih.gov/pubmed/29186264 http://dx.doi.org/10.1590/abd1806-4841.20175716 |
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author | Sanches, Sarah Rebellato, Priscila Regina Orso Fabre, Andréa Buosi de Campos, Giovana Liz Marioto |
author_facet | Sanches, Sarah Rebellato, Priscila Regina Orso Fabre, Andréa Buosi de Campos, Giovana Liz Marioto |
author_sort | Sanches, Sarah |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-5514592 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | Sociedade Brasileira de Dermatologia |
record_format | MEDLINE/PubMed |
spelling | pubmed-55145922017-07-24 Do you know this syndrome? Clouston syndrome Sanches, Sarah Rebellato, Priscila Regina Orso Fabre, Andréa Buosi de Campos, Giovana Liz Marioto An Bras Dermatol Syndrome in Question 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. Sociedade Brasileira de Dermatologia 2017 /pmc/articles/PMC5514592/ /pubmed/29186264 http://dx.doi.org/10.1590/abd1806-4841.20175716 Text en ©2017 by Anais Brasileiros de Dermatologia http://creativecommons.org/licenses/by-nc/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License which permits unrestricted non-commercial use, distribution, and reproduction in any medium provided the original work is properly cited. |
spellingShingle | Syndrome in Question Sanches, Sarah Rebellato, Priscila Regina Orso Fabre, Andréa Buosi de Campos, Giovana Liz Marioto Do you know this syndrome? Clouston syndrome |
title | Do you know this syndrome? Clouston syndrome |
title_full | Do you know this syndrome? Clouston syndrome |
title_fullStr | Do you know this syndrome? Clouston syndrome |
title_full_unstemmed | Do you know this syndrome? Clouston syndrome |
title_short | Do you know this syndrome? Clouston syndrome |
title_sort | do you know this syndrome? clouston syndrome |
topic | Syndrome in Question |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5514592/ https://www.ncbi.nlm.nih.gov/pubmed/29186264 http://dx.doi.org/10.1590/abd1806-4841.20175716 |
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