A case of hereditary angioedema with acute abdominal pain during postpartum

A 35-year-old woman had often experienced acute edema of the extremities with spontaneous improvement after 3-4 days from around eight years of age. Laboratory findings showed low levels of C4 and C1-INH activation, indicating hereditary angioedema (HAE). She had hesitated to be treated with human C1-INH concentrate and had been treated with corticosteroid injections when she complained abdominal symptoms. She was referred to our department after she moved for her baby's delivery. Two weeks after delivery, she presented with severe abdominal pain along with nausea and vomiting and made an emergency visit to our hospital. Plain CT findings of the abdomen showed ascites and marked intestinal edema. Her abdominal pain was intense. After obtaining informed consent, we administered human C1-INH concentrate, which dramatically improved her symptoms within about 30 minutes. As the patient had no family history of HAE, we performed genetic analysis and found a C1-INH gene point mutation (M470K). Since patients with HAE often visit hospitals complaining of acute abdominal pain, medical staff should share HAE information with each other to provide appropriate patient care.