This inherited condition is caused by deficiency of the substance known as C1 esterase inhibitor. The deficiency results in recurrent swelling of external and internal tissues that are treated by intravenous infusion of the inhibitor. The condition may result in sudden death from acute swelling of the larynx1, 2.
Recurrence of non-hereditary episodes of angioedema have been reported in streptococcal tonsillitis3.
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