Clinical PearlA patient with idiopathic angioedema presenting with superior vena cava syndrome and lymphedema
Section snippets
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
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Cited by (4)
Superior Vena Cava Syndrome
2021, Actas Dermo-SifiliograficasIf not angioedema, what is it? Diagnostic approach to facial edema
2024, JDDG - Journal of the German Society of DermatologyRadiotherapy in the setting of hypersensitivity syndromes
2020, Breast JournalChronic urticaria associated with occult papillary thyroid carcinoma and dramatic improvement after total thyroidectomy: A retrospective study from Turkey
2019, Journal of Investigational Allergology and Clinical Immunology
Disclosures: Authors have nothing to disclose.
Funding Source: This work was supported by grant T32 AI 007469 from the National Institutes of Health (Dr Eastman).