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Anaphylaxis due to foreign antigens has been known for over 100 years. Idiopathic anaphylaxis was identified in humans less than 2 decades ago. It occurs without any external antigen and has been classified relative to type (angioedema or generalised) and frequency (infrequent or frequent). Therapeutic regimens have been established that result in control and then remission in most cases. The number of individuals with idiopathic anaphylaxis in the US is estimated to be about 30 000.
Theoretical explanations for the underlying mechanisms have been postulated and are under study. A variant, undifferentiated somatoform idiopathic anaphylaxis, mimics idiopathic anaphylaxis but is not an organic disease. However, this variant can account for high medical costs with no benefit until the appropriate diagnosis is made.
KeywordsMast Cell Adis International Limited Angioedema Allergy Clin Immunol Mastocytosis
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