Hereditary hemorrhagic angiomatosis (Randu-Osler-Weber disease): causes, symptoms, diagnosis, treatment
Last reviewed: 23.04.2024
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Angiomatosis is hereditary hemorrhagic (Rundu-Osler-Weber disease syn.) - hereditary autosomal dominant disease, the gene locus is 9q33-34. It is characterized by vascular anomalies in the form of telangiectasias, arachnid vascular nevi, angiomatic-like elements located mainly on the face skin, in the oral cavity, digestive tract and other organs, which leads to frequent bleeding, especially nasal.
Pathomorphology of angiomatosis of hereditary hemorrhagic (Randyu-Osler-Weber disease). In the dermis, there are sinusoidal structures lined with a flattened endothelium, surrounded by connective tissue layers. Dystrophic changes are observed in the surrounding tissue.
Histogenesis of angiomatosis of hereditary hemorrhagic (Rundu-Osler-Weber disease). Increased bleeding is associated with an increase in the content of the plasminogen activator in the telangiectasis zone, which leads to an increase in fibrinolytic activity in the pericapillary tissue. In most cases, the defect of the vascular walls is localized in the venules, but larger vessels may be involved.
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