[Rendu-Osler-Weber disease. Contribution of ten cases]

Ten new cases of hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber's disease) are presented. Five of them were detected among 30,000 patients attending a Service of Internal Medicine; the remainder five, were detected while conducting the family study. Epistaxis (5 cases) and hidden (2 cases) and evident (1 case) digestive hemorrhage were the most frequent clinical signs. All patients presented telangiectasia affecting lips. We observed edemas in nasal fossas (5 cases), hand fingers (6 cases), tongue (2 cases) and face (1 case), as well as in gastroduodenal mucosa in the three patients undergoing endoscopic study. We did not observed hemostasis disorders, except for moderate thrombocytosis in two patients and microcytic anemia in three patients. The relevance of clinical evaluation is stressed, because all patients presented oral-facial telangiectasia.