Anais Brasileiros de Dermatologia (Jul 2014)

Syndrome in Question

  • Sheila Itamara Ferreira do Couto Meireles,
  • Sônia Maria Fonseca de Andrade,
  • Maria Fernanda Gomes,
  • Fernanda Aalmeida Nunes Castro,
  • Antonio José Tebcherani

DOI
https://doi.org/10.1590/abd1806-4841.20142646
Journal volume & issue
Vol. 89, no. 4
pp. 679 – 680

Abstract

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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.

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