REFRACTORY ABDOMINAL WALL ANGIOEDEMA IN A YOUNG FEMALE WITH KNOWN HEREDITARY ANGIOEDEMA

Abstract

Introduction Hereditary angioedema (HAE) is a rare disorder characterized by recurrent episodes of angioedema. The abdomen is involved in 50% of attacks and often resembles an acute abdomen. We present an unusual cause of refractory abdominal wall angioedema. Case Description 37-year-old female with known HAE (low C4 and C1 esterase inhibitor levels) presented to the emergency room with acute left-sided abdominal pain and nausea unresponsive to icatibant. She was previously controlled on C1 esterase inhibitor every other day and twice monthly icatibant. Over the previous three months, her abdominal pain had increased. Her exam revealed left flank edema. An abdominal/pelvic CT demonstrated an 8 x 8 cm cavitary mass arising from the descending colon involving the abdominal wall. Biopsy confirmed adenocarcinoma. She underwent resection and end colostomy. She developed an intraperitoneal abscess with fistulization to her left abdominal wall, causing daily flares incompletely controlled despite C1 esterase inhibitor 2000 units daily and icatibant TID. Chemotherapy and abscess drainage led to some improvement. She passed away six months later. Discussion HAE causes abdominal symptoms like colic, nausea, vomiting and diarrhea, however there is limited data on the management of attacks resembling an acute abdomen in patients with known HAE. To the best of our knowledge, cancer has not been previously reported as a known trigger of HAE attacks. This case highlights the importance of finding an explanation in patients with previously well-controlled HAE whose disease suddenly changes. Both the patient and her physician attributed her symptoms to HAE, which delayed the diagnosis of malignancy.