Hereditary angioedema is, so that we all understand each other, a chronic disease of autosomal dominant inheritance, which is complicated to explain, but I will try. It is caused by a deficiency or dysfunction of the complement system, specifically of the C1 inhibitor, a key protein in the inflammation cascade. When it is missing, it does not produce the necessary braking of inflammation.
It should be said that its onset is more frequent during childhood or adolescence. Whether due to emotional stress or minimal stimuli, such as minor trauma (including dental trauma, piercings, etc.), viral diseases, pregnancy, exposure to cold or the ingestion of certain foods, major inflammations can be triggered, leading to the outflow of fluid from the vessels. Then what we call angioedema occurs when it happens deep in the skin.
Laryngeal edema is usually present, causing stridor and sometimes death. In addition, digestive tract involvement is frequent. Pruritus, habonosis lesions and closure of the lower airways are not usually found.
Defects in complement factors are also present in known diseases such as systemic lupus erythematosus, common variable immunodeficiency or hemolytic uremic syndrome.
Several types are distinguished according to the cause of the alteration, so the diagnosis must be made by a specialist: allergist, dermatologist, internist, etc., so that the appropriate preventive or acute treatment can be indicated.