Syndrome in Question

Anais Brasileiros de Dermatologia

Sheila Itamara Ferreira Do Couto Meireles¹  Sônia Maria Fonseca De Andrade¹  Maria Fernanda Gomes¹  Fernanda Aalmeida Nunes Castro¹  Antonio José Tebcherani¹ 

关键词: Arteriovenous fistula;    Epistaxis;    Gastrointestinal hemorrhage;    Skin abnormalities;    Skin diseases;    vascular;   

DOI  :  10.1590/abd1806-4841.20142646

来源: SciELO

【 摘 要 】

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.

【 授权许可】

CC BY-NC   
 All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License