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Angiomatosis hereditary hemorrhagic (Randu-Osler-Weber disease): causes, symptoms, diagnosis, treatment
Medical expert of the article
Last reviewed: 07.07.2025
Hereditary hemorrhagic angiomatosis (syn. Rendu-Osler-Weber disease) is a hereditary autosomal dominant disease, gene locus - 9q33-34. It is characterized by vascular anomalies in the form of telangiectasias, spider-like vascular nevi, angioma-like elements located mainly on the skin of the face, in the oral cavity, digestive tract and other organs, which leads to frequent bleeding, especially nosebleeds.
Pathomorphology of hereditary hemorrhagic angiomatosis (Rendu-Osler-Weber disease). Sinus-like structures lined with flattened endothelium and surrounded by connective tissue layers are found in the dermis. Dystrophic changes are observed in the surrounding tissue.
Histogenesis of hereditary hemorrhagic angiomatosis (Rendu-Osler-Weber disease). Increased bleeding is associated with an increase in the content of plasminogen activator in the telangiectasia zone, which leads to an increase in fibrinolytic activity in the pericapillary tissue. In most cases, the vascular wall defect is localized in venules, but larger vessels may also be involved.
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