Abstract
Hereditary angioedema (HAE) is a rare autosomal dominant disease, accompanied by symptoms of angioedema caused by not clear cause or provocation by various triggers. Angioedema is recurrent, subcutaneous or submucosal, without pruritus and urticaria, dough-like and if it is located in the larynx, it can be life-threatening.
Attacks can be localized in the digestive tract and cause severe vomiting, abdominal pain and diarrhea. Angioedema attacks have sometimes prodroms: tingling, pain, tension, salivation.
The most common trigger is stress, mechanical trauma, infection, hormonal changes etc. In children mostly machanical trauma and infection.
Angioedema without treatment persists 3-5 day, the beginning is typically in puberty, it is a negative family history. According to the last consensus on the diagnosis and treatment of HAE we differentiate several types of HAE, in differential diagnosis we have to distinguish acquired angioedema.
Treatment of HAE is causal in the HAE attack (substitution treatment of C1INH or inhibiton of receptors for bradykinin). Prophylactic treatment with attenuated androgens or tranexamic acid is indicated in patients with frequent attacks.
Substitution treatment is indicated before the expected trigger mechanism (stomatological and surgical procedures, delivery, etc.). HAE should be avoided in all patients with non-allergic angioedema or unclear digestive problems, thus avoiding the risk of HAE attacks in the larynx.
The patient also benefits from psychological support due to permanent stress.