Urticarial vasculitis is a subtype of leukocytoclastic vasculitis. It may present as part of a systemic disease, arise in association with an antigen exposure, or be idiopathic in nature. An association between urticarial vasculitis and systemic lupus erythematosus has been hypothesized because some clinical manifestations of disease overlap and Clq autoantibodies may be present in both diseases. Normocomplementemic patients have a better prognosis and usually have minimal or no systemic involvement. One the other hand, hypocomplementemic patients are more likely to have more severe multiorgan involvement. In idiopathic urticarial vasculitis, the laboratory studies of diagnostic significance are elevation of erythrocyte sedimentation rate and reduction of serum complement. Confirmation of the diagnosis requires obtaining a biopsy specimen of the skin lesion, however. Response to treatment is variable, and a wide variety of therapeutic agents may be efficacious. Initial recommendations for treatment of urticarial vasculitis manifest only as nonnecrotizing skin lesions include antihistamines, dapsone, colchicine, hydroxychloroquine or indomethacin, but corticosteroids are often required. With necrotizing skin lesions or visceral involvement, corticosteroids are usually indicated. Refractoriness to corticosteroids or corticosteroid morbidity may necessitate treatment with other immunosuppressive agents, such as azathioprine or cyclophosphamide.