Urticarial syndromes and autoinflammation

Abstract

Core Messages: Recent genetic studies of urticaria have revealed distinct molecular pathways underlying different types of urticaria, and have defined several urticarial syndromes associated with autoinflammation. Although the cutaneous presentation of urticarial syndromes is strikingly similar, many of these syndromes involve diverse features of systemic inflammation and multiorgan involvement. These autoinflammatory syndromes lack the typical autoreactive lymphocytes or autoantibodies seen in autoimmune disease, instead marked by aberrant cytokine regulation. To date, the genetic basis for six autoinflammatory syndromes with urticaria has been elucidated and are reviewed here. Therapies targeting cytokine pathways have provided promising treatment for many of these autoinflammatory diseases. © 2010 Springer-Verlag Berlin Heidelberg.