P179 Delayed diagnosis of hereditary angioedema

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Johnson City, TN; 2. Jonesborough, TN; 3. Elizabethton, TN. Introduction: Type II Hereditary angioedema (HAE) is character- ized by dysfunctional C1-inhibitor ...
Abstracts: Poster Sessions / Ann Allergy Asthma Immunol 119 (2017) S17eS96

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P178 SEVERE CHRONIC IDIOPATHIC URTICARIA AND ANGIOEDEMA WITH AN ELEVATED ANTI-IGE IGG LEVEL J. Brooks, F. Hsu, A. Helgeson*, New Haven, CT. Introduction: Chronic idiopathic urticaria (CIU) is characterized by pruritic urticarial lesions, but the underlying mechanism is not understood. Proposed etiologies have implicated autoantibodies, cellular mast cell and/or basophil defects. The symptoms experienced by patients with CIU may cause significant morbidity, especially if facial and oropharyngeal angioedema occur. Here, we discuss a patient with CIU, recurrent angioedema and an elevated anti-IgE IgG. Case Presentation: A 42-year-old female, with history of hypertension and migraine headaches, presented with daily hives and facial swelling. There were no identifiable triggers and no symptom improvement with epinephrine and oral steroids. Her C4 level was 14 mg/dL (normal 16-47) but C1 inhibitor was normal. Her IgG was 537 mg/dL (normal 700-1600), IgM was 263 mg/dL (normal 40230), with low class switched B cells. She had unremarkable ImmunoCAP allergy testing, a normal chronic urticaria index, tryptase, and normal bone marrow biopsy. High dose antihistamines and steroids, montelukast, doxepin, and cyclosporine provided only modest symptomatic improvement. Brief trials of omalizumab, dapsone, ketotifen, and IVIG provided no additional benefit. After additional hospitalizations, once requiring intubation, repeat studies identified an anti-IgE IgG of 1223 ng/mL (normal