Do you know this syndrome? Clouston syndrome
Autor(a) principal: | |
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Data de Publicação: | 2017 |
Outros Autores: | , , |
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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Anais brasileiros de dermatologia (Online) |
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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 |
_version_ |
1752126422122496000 |