hereditary hemorrhagic telangiectasia

hereditary hemorrhagic telangiectasia, a vascular anomaly, inherited as an autosomal-dominant trait, characterized by hemorrhagic telangiectasia of the skin and mucosa. Small red-to-violet lesions are found on the lips, oral and nasal mucosa, tongue, and tips of fingers and toes. The thin, dilated vessels may bleed spontaneously or as a result of only minor trauma, and this condition becomes progressively more severe. Bleeding from superficial lesions is often profuse and may result in severe anemia. No specific treatment is known, but accessible bleeding lesions may be treated with pressure, styptics, and topical hemostatics. Transfusions may be indicated for acute hemorrhage, and iron deficiency anemia may require continuous treatment. Also called hemorrhagic familial angiomatosis, Osler-Weber-Rendu syndrome, Rendu-Osler-Weber syndrome.