What's Your Diagnosis? Angioedema in the ED

Welcome to this month’s What’s Your Diagnosis Challenge!

But before we begin, check to see if you got last month’s case on Advances in Cardiac Resuscitation in the Emergency Department correct.

Case Presentation: Angioedema in the Emergency Department: An Evidence-Based Update

A 23-year-old man presents by EMS with abdominal pain…

The patient reports that for the past 4 hours he has had generalized abdominal pain that is sharp, constant, and increasing in intensity. He has associated nausea and nonbloody emesis. He denies fever, back pain, urinary complaints, chest pain, shortness of breath, or penile discharge. He denies recent NSAID, antibiotic, or drug or alcohol use, as well as recent travel or sick contacts. He states his father’s lips occasionally swell but does not know any other details of the condition. He does report having multiple similar occurrences of this abdominal pain, but with negative lab testing and imaging.
His vital signs are: temperature, 36.6C°; heart rate, 112 beats/min; blood pressure, 104/70 mm Hg; respiratory rate, 22 breaths/min; and oxygen saturation, 100% on room air.
His abdomen is soft, moderately distended, and diffusely tender to palpation without guarding or rebound. His genital exam is unremarkable. You are concerned for a surgical abdomen, but the recurrent nature of the presentation makes you suspicious that something else is going on…

Case Conclusion

You had a wide differential for this patient with recurrent abdominal pain, including appendicitis, testicular torsion, mesenteric ischemia, intussusception, small-bowel obstruction, sexually transmitted infections, pyelonephritis, liver disease, and gastroparesis, among others. You found the recurrent nature of his symptoms, coupled with a family history of what appeared to be angioedema interesting.

You noted that, in spite of previous negative workups, his presentation was concerning for acute emergent pathology, so you obtained repeat laboratory tests and ordered a CT of his abdomen and pelvis with IV contrast, in which you noted a leukocytosis and bowel wall edema without ischemia. His examination was concerning, so you consulted surgery, and they recommended observation to medicine and further diagnostic evaluation.

You remembered that HAE can present with bowel edema, so you added on complement and C1-INH levels and admitted him to the hospital. Upon chart review, you noted the patient had low C1-INH levels and was currently being treated with C1-INH, with improved symptoms.