Do you know this syndrome? Clouston syndrome

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.

Saved in:
Bibliographic Details
Main Authors: Sanches,Sarah, Rebellato,Priscila Regina Orso, Fabre,Andréa Buosi, Campos,Giovana Liz Marioto de
Format: Digital revista
Language:English
Published: Sociedade Brasileira de Dermatologia 2017
Online Access:http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017000300417
Tags: Add Tag
No Tags, Be the first to tag this record!
id oai:scielo:S0365-05962017000300417
record_format ojs
spelling oai:scielo:S0365-059620170003004172017-09-13Do you know this syndrome? Clouston syndromeSanches,SarahRebellato,Priscila Regina OrsoFabre,Andréa BuosiCampos,Giovana Liz Marioto de Palmoplantar keratoderma Ectodermal dysplasia Hypotrichosis 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.info:eu-repo/semantics/openAccessSociedade Brasileira de DermatologiaAnais Brasileiros de Dermatologia v.92 n.3 20172017-06-01info:eu-repo/semantics/reporttext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017000300417en10.1590/abd1806-4841.20175716
institution SCIELO
collection OJS
country Brasil
countrycode BR
component Revista
access En linea
databasecode rev-scielo-br
tag revista
region America del Sur
libraryname SciELO
language English
format Digital
author Sanches,Sarah
Rebellato,Priscila Regina Orso
Fabre,Andréa Buosi
Campos,Giovana Liz Marioto de
spellingShingle Sanches,Sarah
Rebellato,Priscila Regina Orso
Fabre,Andréa Buosi
Campos,Giovana Liz Marioto de
Do you know this syndrome? Clouston syndrome
author_facet Sanches,Sarah
Rebellato,Priscila Regina Orso
Fabre,Andréa Buosi
Campos,Giovana Liz Marioto de
author_sort Sanches,Sarah
title Do you know this syndrome? Clouston syndrome
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
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.
publisher Sociedade Brasileira de Dermatologia
publishDate 2017
url http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962017000300417
work_keys_str_mv AT sanchessarah doyouknowthissyndromecloustonsyndrome
AT rebellatopriscilareginaorso doyouknowthissyndromecloustonsyndrome
AT fabreandreabuosi doyouknowthissyndromecloustonsyndrome
AT camposgiovanalizmariotode doyouknowthissyndromecloustonsyndrome
_version_ 1756412569431572480