HEREDITARY hemorrhagic telangiectasia (Rendu-Osler-Weber disease) is a disorder in which tiny arteriovenous aneurysms may present in virtually every visceral organ and on every body surface. There are vascular sinuses lined by a thin layer of endothelium interposed between arteries and veins. Because of the lack of elastic tissue in their walls, the vessels, when injured, bleed severely before stopping. The cause is unknown. The disorder may be passed as a simple dominant trait from either parent to both male and female children. There is a tendency for new telangiectases to appear as the person grows older.
Although the telangiectases may effect any body structure, it is the nose that bleeds most commonly, and epistaxis is the bane of the patient's life. A typical patient usually starts to have nosebleeds about puberty. The nosebleeds, often only a few drops every day but sometimes a quart or more at a time, weaken
Saunders WH. Hereditary Hemorrhagic TelangiectasiaControl of Nosebleeds by Septal Dermoplasty. JAMA. 1960;174(15):1972–1974. doi:10.1001/jama.1960.63030150005020
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