Urticarial Vasculitis

• Urticarial vasculitis is a multisystem disease characterized by cutaneous lesions resembling urticaria, except that wheals persist for >24 hours. Urticaria like (i.e., edematous plaques and wheals), occasionally indurated, erythematous, circumscribed (Fig. 14-58); lesions may be associated with itching, burning, stinging sensation, pain, tenderness. Occasionally with angioedema. Eruption occurs in transient crops, usually lasting >24 hours and up to 3 to 4 days. They change shape slowly, often reveal purpura on blanching (glass slide), and resolve with a yellowish-green color and hyperpigmentation.
• Fever, arthralgia, and elevated ESR. Other symptoms: Nausea, abdominal pain. Cough, dyspnea, chest pain, and hemoptysis. Pseudotumor cerebri. Cold sensitivity. Renal involvement: diffuse glomerulonephritis.
• The syndrome is often accompanied by various degrees of extracutaneous involvement. Extracutaneous manifestations: joints (70%), GI tract (20 to 30%), CNS (>10%), ocular system (>10%), kidneys (10 to 20%), lymphadenopathy (5%).
• Thought to be an immune complex disease, similar to hypersensitivity vasculitis (see Hypersensitivity Vasculitis (HV)). May be symptom of SLE; in serum sickness, hepatitis B; idiopathic.
• Laboratory: Leukocytoclastic vasculitis; microhematuria, proteinuria (10%); hypocomplementemia (70%).
• Most often this syndrome has a chronic (months to years) but benign course. Episodes recur over periods ranging from months to years. Renal disease recur over periods ranging from months to years. Renal disease occurs only in hypocomplementemic patients.
• Management: H₁ and H₂ blockers (doxepin [10 mg twice daily to 25 mg three times daily]) plus cimetidine [300 mg three times daily]/ranitidine [150 mg twice daily]) plus a nonsteroidal anti-inflammatory agent (indomethacin [75 to 200 mg/d]/ibuprofen [1600 to 2400 mg/d]/naprosyn [500 to 1000 mg/d]). Colchicine, 0.6 mg two or three times daily or dapsone, 50 to 150 mg/d. Prednisone; azathioprine, cyclophosphamide; plasmapheresis. TNF-α blockers.