Hereditary angioedema (HAE) is a rare, but potentially life-threatening condition, clinically characterized by recurrent and self-limiting episodes of swelling which affect the skin, gastrointestinal tract and upper airways, and are caused by a lack of complement-C1-inhibitor (C1-INH). Within the past ten years, two Danish HAE patients have died from laryngeal oedema. We report two cases that illustrate symptoms and differential diagnostic problems posed by this disease, and the importance of an effective treatment strategy.
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