Rendu-Osler-Weber syndrome: dermatological approach

Rendu-Osler-Weber syndrome: dermatological approach

Abstract The Rendu-Osler-Weber syndrome is a rare systemic fibrovascular dysplasia, recognized by mucocutaneous telangiectasias, arteriovenous malformations, epistaxis and family history. Recurrent bleeding, hypoxemia, congestive heart failure, portosystemic encephalopathy, and symptoms related to angiodysplasia of the central nervous system may occur. Since the treatment is based on supportive measures, early recognition is of utmost importance. This article reports the case of a 53-year-old male patient who presented telangiectasias on fingers, oral cavity and nasal mucosa for 10 years, with a history of recurrent epistaxis of varying severity since childhood. Mother, sister and daughter have similar lesions.

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Main Authors: Barbosa,Aline Blanco, Hans Filho,Günter, Vicari,Carolina Faria dos Santos, Medeiros,Marcelo Zanolli, Couto,Daíne Vargas, Takita,Luiz Carlos
Format: Digital revista
Language:English
Published: Sociedade Brasileira de Dermatologia 2015
Online Access:http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962015000700226
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spelling oai:scielo:S0365-059620150007002262015-11-19Rendu-Osler-Weber syndrome: dermatological approachBarbosa,Aline BlancoHans Filho,GünterVicari,Carolina Faria dos SantosMedeiros,Marcelo ZanolliCouto,Daíne VargasTakita,Luiz Carlos Epistaxis Genetics Telangiectasia hereditary hemorrhagic Abstract The Rendu-Osler-Weber syndrome is a rare systemic fibrovascular dysplasia, recognized by mucocutaneous telangiectasias, arteriovenous malformations, epistaxis and family history. Recurrent bleeding, hypoxemia, congestive heart failure, portosystemic encephalopathy, and symptoms related to angiodysplasia of the central nervous system may occur. Since the treatment is based on supportive measures, early recognition is of utmost importance. This article reports the case of a 53-year-old male patient who presented telangiectasias on fingers, oral cavity and nasal mucosa for 10 years, with a history of recurrent epistaxis of varying severity since childhood. Mother, sister and daughter have similar lesions.info:eu-repo/semantics/openAccessSociedade Brasileira de DermatologiaAnais Brasileiros de Dermatologia v.90 n.3 suppl.1 20152015-06-01info:eu-repo/semantics/reporttext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962015000700226en10.1590/abd1806-4841.20152563
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country Brasil
countrycode BR
component Revista
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databasecode rev-scielo-br
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region America del Sur
libraryname SciELO
language English
format Digital
author Barbosa,Aline Blanco
Hans Filho,Günter
Vicari,Carolina Faria dos Santos
Medeiros,Marcelo Zanolli
Couto,Daíne Vargas
Takita,Luiz Carlos
spellingShingle Barbosa,Aline Blanco
Hans Filho,Günter
Vicari,Carolina Faria dos Santos
Medeiros,Marcelo Zanolli
Couto,Daíne Vargas
Takita,Luiz Carlos
Rendu-Osler-Weber syndrome: dermatological approach
author_facet Barbosa,Aline Blanco
Hans Filho,Günter
Vicari,Carolina Faria dos Santos
Medeiros,Marcelo Zanolli
Couto,Daíne Vargas
Takita,Luiz Carlos
author_sort Barbosa,Aline Blanco
title Rendu-Osler-Weber syndrome: dermatological approach
title_short Rendu-Osler-Weber syndrome: dermatological approach
title_full Rendu-Osler-Weber syndrome: dermatological approach
title_fullStr Rendu-Osler-Weber syndrome: dermatological approach
title_full_unstemmed Rendu-Osler-Weber syndrome: dermatological approach
title_sort rendu-osler-weber syndrome: dermatological approach
description Abstract The Rendu-Osler-Weber syndrome is a rare systemic fibrovascular dysplasia, recognized by mucocutaneous telangiectasias, arteriovenous malformations, epistaxis and family history. Recurrent bleeding, hypoxemia, congestive heart failure, portosystemic encephalopathy, and symptoms related to angiodysplasia of the central nervous system may occur. Since the treatment is based on supportive measures, early recognition is of utmost importance. This article reports the case of a 53-year-old male patient who presented telangiectasias on fingers, oral cavity and nasal mucosa for 10 years, with a history of recurrent epistaxis of varying severity since childhood. Mother, sister and daughter have similar lesions.
publisher Sociedade Brasileira de Dermatologia
publishDate 2015
url http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962015000700226
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AT vicaricarolinafariadossantos renduoslerwebersyndromedermatologicalapproach
AT medeirosmarcelozanolli renduoslerwebersyndromedermatologicalapproach
AT coutodainevargas renduoslerwebersyndromedermatologicalapproach
AT takitaluizcarlos renduoslerwebersyndromedermatologicalapproach
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