Rendu-Osler-Weber syndrome: dermatological approach

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 angiodyspl...

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Published inAnais brasileiros de dermatología Vol. 90; no. 3 Suppl 1; pp. 226 - 228
Main Authors Barbosa, Aline Blanco, Hans Filho, Günter, Vicari, Carolina Faria dos Santos, Medeiros, Marcelo Zanolli, Couto, Daíne Vargas, Takita, Luiz Carlos
Format Journal Article
LanguageEnglish
Published Spain Sociedade Brasileira de Dermatologia 01.06.2015
Subjects
Case Report
DERMATOLOGY
Epistaxis
Epistaxis - pathology
Esophageal Diseases - pathology
Genetic Diseases, Inborn - pathology
Genetics
hereditary hemorrhagic
Humans
Male
Middle Aged
Stomach Diseases - pathology
Syndrome
Telangiectasia
Telangiectasia, Hereditary Hemorrhagic - pathology
Tongue Diseases - pathology
Epistaxis
Genetics
hereditary hemorrhagic
Telangiectasia
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Summary: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.
Bibliography:ObjectType-Case Study-2
SourceType-Scholarly Journals-1
ObjectType-Feature-4
content type line 23
ObjectType-Report-1
ObjectType-Article-3
Conflict of Interest: None.
ISSN:0365-0596
1806-4841
1806-4841
0365-0596
DOI:10.1590/abd1806-4841.20152563