Syndrome in question

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,muco-cutaneous telangiectasia,visceral arteriovenous malformation and positive family history. there may be hema-tologic,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. © 2014 by anais brasileiros de dermatologia.