Episodes of recurrent urticaria and angioedema may be a clinical manifestation of cutaneous necrotizing venulitis. Known as urticarial vasculitis, this edematous form of necrotizing venulitis occurs in patients with serum sickness, connective tissue disorders, hematologic malignant conditions, an IgMKM component, infections, or physical urticarias after the administration of therapeutic agents, and as an idiopathic disorder. The skin lesions appear as erythematous, occasionally indurated wheals which may contain foci of purpura. Although the individual urticarial lesions may last for less than 24 hours, they often persist up to 3–5 days. The episodes of urticaria are chronic; approximately 70% of the afflicted individuals are women. General features include fever, malaise, and myalgia. Specific organ involvement includes enlargement of the lymph nodes, liver, and spleen and involvement of the synovia, kidneys, gastrointestinal tract, respiratory tract, eyes, and central nervous system. In the treatment of urticarial vasculitis there are no double‐blind, placebo‐controlled trials. Reports exist of the treatment of patients with H1 antihistamines, nonsteroidal anti‐inflammatory agents, colchicine, hydroxychloroquine, dapsone, prednisone, azathioprine, methotrexate, cyclophosphamide, and intramuscular gold therapy.