Urticarial Vasculitis Syndrome

Abstract

More than twenty years ago, a group of patients was described with the clinical presentation of chronic urticaria and with necrotizing cutaneous vasculitic changes on histology (McDuffie et al. 1973, Sissons et al. 1974, Soter et al. 1974, Agnello et al. 1975 and 1976). This combination is relatively rare and encompasses a wide spectrum of clinical symptoms and laboratory changes. It is furthermore characterized by relative resistance to therapy. The syndrome is classified into idiopathic types (primary urticarial vasculitis) and secondary ones in association with severe general diseases such as systemic lupus erythematosus, hepatitis B and neoplasias. A differentiation of urticarial vasculitis from ordinary urticaria is thus of great importance, particularly regarding treatment and prognosis. On the basis of laboratory changes, the syndrome is further classified into hypocomplementemic and normocomplementemic vasculitis.