Abstract

CRUZ RODRIGUEZ, Javier; JIMENEZ LOPEZ, Mildrey  and  HIDALGO MESA, Carlos. Rendú-Osler-Weber's syndrome: Apropos of a case. Rev Cubana Med Gen Integr [online]. 2007, vol.23, n.1. ISSN 0864-2125.

The hereditary hemorrhagic telangiectasia or Rendú-Osler-Weber syndrome is a vascular disorder inherited as a dominant autosomal trait, characterized by telangiectasis in skin and mucous membranes. The thin and dilated vessels may bleed spontaneously or as result of mild traumas. The lesions are detected during the first years of life and it is usual to observe recurrent epistaxis in children. At the 10 years of age, 50 % of the patients have already suffered from some gastrointestinal hemorrhage, but they are not severe until the fourth decade of life. In this paper, a 56-year-old female patient with typical characteristics of this disease that was seen at the office is presented.

Keywords : Hereditary hemorrhagic telangiectasia; hereditary disease; Rendú-Osler-Weber's syndrome.