angioedemaCase: A 24 year old male presents with right sided lip swelling that began several hours ago. This is the second time he has had this type of swelling. His mother has also had this before. He currently has no urticaria, dyspnea, wheezing, or stridor. What is the cause of this patient’s symptoms?

Poll Results

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C1 inhibitor deficiency

Explanation

Angioedema is characterized by non-pitting edema of the dermis and subcutaneous layers of the extremities, abdomen, genitourinary tract, tongue, lips, face, or larynx. The cause of this patient’s presentation is hereditary angioedema, an autosomal dominant disease caused by a decrease in C1 inhibitor enzyme.1

This patient’s unilateral lip swelling progressed rapidly.

angioedema intubated

Airway management is the most important aspect of emergency department management. Fiberoptic nasopharyngeal laryngoscopy is indicated when hoarseness, odynophagia, voice change, or dyspnea are present, as these signs may represent significant airway edema.2

Though commonly given, antihistamines, corticosteroids, and epinephrine are not effective for treating non-allergic angioedema. Effective therapies for hereditary angioedema include:2

TherapyMechanism
Berinert/CinryzeHuman plasma derived C1 esterase inhibitor
EcallantideReversible inhibitor of kallikrien, which produces bradykinin from kininogen
FFPNot FDA approved. Contains ACE (kininase II) which degrades bradykinin
IcatibantSelective bradykinin B2 receptor antagonist
RuconestRecombinant human C1 inhibitor, available in Europe

Master Clinician Bedside Pearls

Christopher I. Doty, MD FAAEM FACEP
Program Director & Vice Chair for Education
Associate Professor of Emergency Medicine
Department of Emergency Medicine
University of Kentucky-Chandler Medical Center
Twitter: @PoppasPearls

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1.
Zuraw B, Banerji A, Bernstein J, et al. US Hereditary Angioedema Association Medical Advisory Board 2013 recommendations for the management of hereditary angioedema due to C1 inhibitor deficiency. J Allergy Clin Immunol Pract. 2013;1(5):458-467. [PubMed]
2.
Lewis L. Angioedema: etiology, pathophysiology, current and emerging therapies. J Emerg Med. 2013;45(5):789-796. [PubMed]
Jeff Riddell, MD

Jeff Riddell, MD

Assistant Professor of Clinical Emergency Medicine
Co-Director, Medical Education Fellowship
LA County + USC Emergency Medicine Residency, Department of Emergency Medicine
Keck School of Medicine of the University of Southern California
Jeff Riddell, MD

@jeff__riddell

emergency physician. teacher. education scholar. thinking about #MedEd thru the lens of digital innovation. LAC+USC, TTCoOp, ALiEM, Essentials of EM