Unique Presentation of Isolated Intestinal Angioedema Without ACE-I Involvement

Abstract

1651_A Figure 1. Diffuse bowel wall thickening involving stomach, small bowel and colon with suggestion of mucosal enhancement and submucosal edema.1651_B Figure 2 No Caption available.Case reports of intestinal angioedema are well documented in the literature. However, the majority of these cases are due to angiotensin-converting enzyme inhibitor (ACE-I) induced angioedema. We present an unusual case of a patient who had isolated intestinal wall angioedema without taking ACE-I and normal C1 inhibitor levels. The inciting event was ingestion of a food allergen, which is not well described in the literature. The patient is a 63 year old female who presented for 1 day history of severe abdominal pain. The patient was in her usual state of health when she drank her daily smoothie. The only addition to her smoothie was parsley. Upon ingestion, she immediately developed severe cramping pain in her abdomen associated with nausea, vomiting, and watery diarrhea. She denied itching or rash. Upon presentation to the hospital, the patient was noted to be tachycardic and tachypneic. The patient admitted to having a similar reaction years ago to ingestion of kiwi fruit. Her physical exam demonstrated non distended abdomen with diffuse tenderness to palpation and normoactive bowel sounds. Her hospital course entailed a 1 day course of antibiotics and anti-emetics. Her diet was advanced and after a 3 day hospital stay was discharged home. Labs were notable for WBC of 18,000, lactate of 5.9. The remainder of the labs including blood cultures, stool analysis, ESR, C-ANCA, P-ANCA, C1-Inhibitor, complement levels were normal. Angioedema is one of the most common allergic disorders that results in hospitalization. Any hollow viscera are prone to this type of temporary swelling. The severity of presentation of intestinal angioedema can be varied, from mild colicky pain to severe vomiting and diarrhea. Most often the inciting agent is ACE- I. This reaction is well documented but still rare with only 21 cases described in the literature. We present a case of isolated intestinal angioedema following an ingested allergen. What is unique is that this sequela occurred in the absence of ACE-I use. Furthermore, the patient presented with isolated intestinal angioedema which is unreported in literature. No signs of systemic anaphylaxis or oral angioedema were noted which is typical in food allergies. Another unique finding was normal C1 inhibitor levels making hereditary angioedema unlikely. In this instance, the mechanism was most likely due to first time parsley ingestion causing IgE mediated mast cell degranulation releasing histamine leading to intestinal swelling.