A growing body of evidence shows that at least 40% of patients with unexplained (idiopathic) chronic urticaria have clinically relevant functional autoantibodies to the high-affinity IgE receptor on basophils and mast cells. The term "autoimmune urticaria" is used for this subgroup of patients presenting with continuous ordinary urticaria. This article reviews the evidence for the autoimmune hypothesis and other nonantibody serum histamine-releasing factors in the etiopathogenesis of urticaria; defines autoimmune urticaria; looks at how autoimmune urticaria fits into existing classifications of urticaria; proposes diagnostic criteria that may be useful to the clinician; and reviews the management implications for patients with this subset of chronic disease.