Estrogen-sensitive hereditary angioedema type II: a case report and review -

Hereditary angioedema (HAE) is a rare autosomal condition due to inherited deficiency of C1 esterase inhibitor (C1INH). Estrogen reduces the concentration of C1INH and trigger angioedema; however, the estrogen sensitivity pattern is variable in HAE. Proper diagnosis and management is essential as this condition sometimes becoming life-threatening due to potential airway obstruction. We reported a 27-year-old woman experienced recurrent episodes of lips and face swellings over the last 4 years. Patient has positive family history of similar recurrent angioedema in her brother and cousin. Laboratory findings revealed normal C3, C4, C1q, and C1INH quantity but low activity (36% decreased). Subsequent visits revealed the intake of oral contraceptive pills and improvement of symptoms after discontinuation. To our knowledge, this is the first case report of HAE type II with estrogen-sensitive pattern in a Saudi woman improved after cessation of oral contraceptive pills.