Angioedema

Excerpt

Angioedema is defined as " subcutaneous tissues and/or submucosal tissues circumscribed non-pitting edema affecting lips, face, neck, and extremities oral cavity, larynx, and gut." It becomes life-threatening when it involves the larynx, while intestinal angioedema is painful and mimics acute abdomen.

Angioedema was first described in 1882 by Quincke, then by Osler in 1888 (hereditary angioedema), and finally in 1963 by Donaldson et al (the role of C1 inhibitor).

Classification of angioedema

Acquired

1. Allergic (histaminergic angioedema) associated with anaphylaxis

2. Non-allergic (non-histaminergic angioedema), presenting isolated or in combination with urticaria

3. Drug-induced, e.g., angiotensin-converting enzyme inhibitors and non-steroidal anti-inflammatory drugs

4. Complement-mediated secondary to acquired deficiency of C1-inhibitor

5. Idiopathic which is subdivided into histaminergic and non-histaminergic

Hereditary forms

1. C1-Inhibitor deficiency divided into type 1 (lack of C1-inhibitor molecule) and typed 2 (dysfunctional C1-inhibitor molecule) with normal C1 inhibitor.