Angioedema due to C1-esterase inhibitor (C1-INH) deficiency can occur as a hereditary disorder or, much more rarely, in several acquired forms. Overall, it is extremely rare, about 50-100 times less common than other types of angioedema. There is a considerable number of undiagnosed cases of the disease. Clinically, angioedema due to C1-INH deficiency is characterized by episodic edemas or swellings of the skin, the gastrointestinal tract and, more rarely, of the airways. Despite their rarity, laryngeal edemas are of great importance because of their considerable mortality rate.

As of April 24, 2021