Idiopathic anaphylaxis

The diagnosis of idiopathic anaphylaxis can be made with reasonable certainty in the absence of defined triggers of mast cell activation. Patients can be classified into frequent or infrequent episodes depending on whether there have been at least two episodes within 2 months or six per year. Multisystem involvement defines idiopathic anaphylaxis-generalized and is contrasted with patients who manifest life-threatening upper airway angioedema. Management includes patient education, self-administration of epinephrine, oral corticosteroids, histamine 1 antagonists, and albuterol. In rare patients not responding to these medications, orally administered cromolyn or ketotifen have provided apparent benefit.