A patient with idiopathic angioedema presenting with superior vena cava syndrome and lymphedema

doi:10.1016/j.anai.2016.12.017

Annals of Allergy, Asthma & Immunology

Volume 118, Issue 3, March 2017, Pages 257-258

https://doi.org/10.1016/j.anai.2016.12.017 Get rights and content

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Clinical Presentation

A 40-year-old woman with a history of 2 episodes of corticosteroid-responsive angioedema without urticaria presented to the allergy clinic for evaluation of recurrent angioedema after a third episode. Her medical history was significant for papillary thyroid carcinoma that required anterior neck dissection and radiation as well as epithelioid hemangioendothelioma of the right internal jugular vein that required resection and graft placement. Her first episode of laryngeal edema occurred while

Supporting Data

Laboratory testing revealed normal serum tryptase (3.9 μg/L), C4 (42 mg/dL), C1 esterase inhibitor (32 mg/dL) levels and function (116%). On physical examination, she had symmetric edema of the submandibular area without facial involvement and diffuse erythema of the neck and face. The symptom duration, incomplete corticosteroid response, and negative in vitro test results indicated that bradykinin- or histamine-mediated angioedema was unlikely. On review of her records, she was prescribed

Clinical Outcome

After confirmation of the presence of thrombosis, the patient underwent thrombolysis, and most of the edema, erythema, and dizziness improved. Follow-up computed tomography 1 month later did not reveal any evidence of recurrent malignant tumors, but she continued to have the sensation of laryngeal fullness. Additional laryngoscopy revealed minimal edema of the bilateral vocal cords. She was also diagnosed with lymphedema of the head and neck as a consequence of her previous surgery and