TYPE II HEREDITARY ANGIOEDEMA PRESENTING WITH RECURRING ABDOMINAL PAIN AND ASCITES: CASE REPORT

Aims: Hereditary angioedema (HAE) is an uncommon disorder of the complement system due to a deficiency or dysfunction of the inhibitor of the first component of complement (C1-INH). Because abdominal symptoms may precede by several years the episodes of subcutaneous tissue swelling that are characteristic of HAE, patients may undergo inappropriate surgical and medical treatment for any of a wide range of presumptive, incorrect diagnoses.

Presentation of Case: A 21-year-old girl presented with history of multiple episodes of abdominal pain, swelling of abdomen and upper extremities and ascites. The patient underwent surgery four times and for several months was treated unsuccessfully as Crohn’s disease. A diagnosis of HAE was made on the patient’s and family’s history and on decreased level of complement component 4 (C4) and decreased activity of C1-INH. C1-INH antigen was normal. According to the decreased serum C1-INH activity and C4 concentration, the patient was finally diagnosed with type II HAE. In the absence of other medications the patient was treated with fresh frozen plasma (FFP).

Conclusion: HAE is a rare cause of abdominal pain; however it needs to be taken as one of the differential diagnosis of various acute abdomens in order to avoid unnecessary surgeries.