1. What are the clinical pictures associated w ith angioedema?
2. What pathophysiologic processes underlie angioedema?
3. How is hereditary angioedema (HAE) diagnosed?
Answers:
1. What are the clinical pictures associated with angioedema?
Angioedema can present with several different clinical pictures. It can include an exaggerated form of urticaria, with itching and swelling of soft tissues that can arise anywhere in the body and appear within a few minutes or over the course of hours. Alternatively, it may involve the bronchial mucosa or the vocal cords, leading to airway obstruction. Other forms of angioedema do not include itching or urticaria. They may be local or may result from trauma. In these cases, the swollen tissues may hurt, but do not itch.
2. What pathophysiologic processes underlie angioedema?
In angioedema that coexists with urticaria, the underlying mechanism is the same as that in anaphylaxis— the activation of mast cells with the release of mast cell mediators, such as histamine. In the setting of angioedema without urticaria, the mechanism may involve mast cells or may be the unbridled activation of the complement and kinin systems because of lack of a major complement control protein, C1 inhibitor(C1 INH).
3. How is hereditary angioedema (HAE) diagnosed?
The clinical clue to HAE is a history of repeated bouts of angioedema without urticaria arising anywhere in the body, such as the face, tongue, and extremities. The airway can be compromised. Some patients experience diffuse abdominal pain and may have had laparotomies at which only bowel edema is found. These lesions do not itch and may be painful. HAE is transmitted as an autosomal dominant trait. Nevertheless, the family history is negative in 50% of the patients and these cases are either due to new mutations or mistaken paternity. The laboratory clues point to the complement system, with a deficiency of complement control proteins at fault.
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