Do you know this syndrome? Clouston syndrome

Detalhes bibliográficos
Autor(a) principal: Sanches,Sarah
Data de Publicação: 2017
Outros Autores: Rebellato,Priscila Regina Orso, Fabre,Andréa Buosi, Campos,Giovana Liz Marioto de
Tipo de documento: Relatório
Idioma: eng
Título da fonte: Anais brasileiros de dermatologia (Online)
Texto Completo: http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017000300417
Resumo: Abstract 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.
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spelling Do you know this syndrome? Clouston syndromePalmoplantar keratodermaEctodermal dysplasiaHypotrichosisAbstract 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 Dermatologia2017-06-01info:eu-repo/semantics/reportinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017000300417Anais Brasileiros de Dermatologia v.92 n.3 2017reponame:Anais brasileiros de dermatologia (Online)instname:Sociedade Brasileira de Dermatologia (SBD)instacron:SBD10.1590/abd1806-4841.20175716info:eu-repo/semantics/openAccessSanches,SarahRebellato,Priscila Regina OrsoFabre,Andréa BuosiCampos,Giovana Liz Marioto deeng2017-09-13T00:00:00Zoai:scielo:S0365-05962017000300417Revistahttp://www.anaisdedermatologia.org.br/https://old.scielo.br/oai/scielo-oai.phpabd@sbd.org.br||revista@sbd.org.br1806-48410365-0596opendoar:2017-09-13T00:00Anais brasileiros de dermatologia (Online) - Sociedade Brasileira de Dermatologia (SBD)false
dc.title.none.fl_str_mv Do you know this syndrome? Clouston syndrome
title Do you know this syndrome? Clouston syndrome
spellingShingle Do you know this syndrome? Clouston syndrome
Sanches,Sarah
Palmoplantar keratoderma
Ectodermal dysplasia
Hypotrichosis
title_short 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_sort Do you know this syndrome? Clouston syndrome
author Sanches,Sarah
author_facet Sanches,Sarah
Rebellato,Priscila Regina Orso
Fabre,Andréa Buosi
Campos,Giovana Liz Marioto de
author_role author
author2 Rebellato,Priscila Regina Orso
Fabre,Andréa Buosi
Campos,Giovana Liz Marioto de
author2_role author
author
author
dc.contributor.author.fl_str_mv Sanches,Sarah
Rebellato,Priscila Regina Orso
Fabre,Andréa Buosi
Campos,Giovana Liz Marioto de
dc.subject.por.fl_str_mv Palmoplantar keratoderma
Ectodermal dysplasia
Hypotrichosis
topic Palmoplantar keratoderma
Ectodermal dysplasia
Hypotrichosis
description Abstract 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.
publishDate 2017
dc.date.none.fl_str_mv 2017-06-01
dc.type.driver.fl_str_mv info:eu-repo/semantics/report
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
format report
status_str publishedVersion
dc.identifier.uri.fl_str_mv http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017000300417
url http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017000300417
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv 10.1590/abd1806-4841.20175716
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv text/html
dc.publisher.none.fl_str_mv Sociedade Brasileira de Dermatologia
publisher.none.fl_str_mv Sociedade Brasileira de Dermatologia
dc.source.none.fl_str_mv Anais Brasileiros de Dermatologia v.92 n.3 2017
reponame:Anais brasileiros de dermatologia (Online)
instname:Sociedade Brasileira de Dermatologia (SBD)
instacron:SBD
instname_str Sociedade Brasileira de Dermatologia (SBD)
instacron_str SBD
institution SBD
reponame_str Anais brasileiros de dermatologia (Online)
collection Anais brasileiros de dermatologia (Online)
repository.name.fl_str_mv Anais brasileiros de dermatologia (Online) - Sociedade Brasileira de Dermatologia (SBD)
repository.mail.fl_str_mv abd@sbd.org.br||revista@sbd.org.br
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