Hereditary Angioedema
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Angioedema is an allergic skin disease characterized by patches of confined swelling involving the skin the layers beneath the skin, the mucous membranes, and sometimes the viscera. Angioedema involves vascular leakage beneath the dermis and subcutis. This response is mediated by vasoactive mediators, such as histamine, serotonin, and kinins, which cause the arterioles to dilate while inducing a brief episode of vascular leakage in the venules.
Hereditary angioedema or HAE is an inherited disease caused by low levels or improper function of the plasma protein C1 inhibitor (C1-INH). C1 inhibitor is part of the complement system, which is part of the immune system. In this disorder, an injury, a viral infection, or stress may trigger attacks of swelling. This problem affects the blood vessels. People with hereditary angioedema can develop rapid swelling of the hands, feet, limbs, face, intestinal tract, larynx, or trachea.
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What is hereditary angioedema? Hereditary angioedema or HAE is an autosomal dominant disorder.
What is angioedema? Angioedema refers to swelling that occurs in the tissue just below the surface of the skin, most often around the lips and eyes.
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