Syndrome in Question
An. bras. dermatol
; 89(4): 679-680, Jul-Aug/2014. graf
Article
in En
| LILACS
| ID: lil-715548
Responsible library:
BR1.1
ABSTRACT
Rendu-Osler-Weber Syndrome also known as Hereditary Hemorrhagic Telangiectasia is a rare systemic fibrovascular dysplasia, with dominant autosomal inheritance. It is characterized by recurrent epistaxis, mucocutaneous telangiectasia, visceral arteriovenous malformation and positive family history. There may be hematologic, neurologic, dermatologic and gastrointestinal complications. Therapy is supportive and aimed at preventing complications. In this article we report a case of Rendu-Osler-Weber in a 64 year-old man, with history of mucocutaneous telangiectasia since the third decade of life, recurrent epistaxis, positive family history and vascular ectasia in the gastrointestinal tract.
Key words
Full text:
1
Collection:
01-internacional
Database:
LILACS
Main subject:
Telangiectasia, Hereditary Hemorrhagic
/
Tongue Diseases
Limits:
Humans
/
Male
Language:
En
Journal:
An. bras. dermatol
Journal subject:
DERMATOLOGIA
Year:
2014
Document type:
Article
Affiliation country:
Brazil