Congenital angiotensin-converting enzyme deficiency presenting as recurrent angioedema of upper airway in adult life.

In summary, a rare case of congenital ACE deficiency is presented. This disorder is of concern to the otolaryngologist because the patient in question had episodes of recurrent upper airway angioedema. Management of angioedema generally consists of self-administered epinephrine because these patients respond poorly to steroids.