Urticarial vasculitis is an eruption of erythematous wheals that clinically resemble urticaria but histologically showchanges of leukocytoclastic vasculitis. Urticarial vasculitis may be divided into normocomplementemic and hypocomplementemic variants. Both subsets can be associated with systemic symptoms (eg, angioedema, arthralgias, abdominal or chest pain, fever, pulmonary disease, renal disease, episcleritis, uveitis). The hypocomplementemic form more often is associated with systemic symptoms and has been linked to connective-tissue disease (ie, systemic lupus erythematosus [SLE]) .1

The pathophysiology of urticarial vasculitis is similar to other forms of cutaneous small vessel leukocytoclastic vasculitis. Urticarial vasculitis is a type III hypersensitivity reaction in which antigen-antibody complexes are deposited in the vascular lumina. This reaction results in complement activation and chemotaxis of neutrophils. These cells release various proteolytic enzymes, such as collagenase and elastase, resulting in damage to the vascular lumina. Some authors have speculated that eosinophils may be involved in the early stages of the vasculitic lesions. Patients with hypocomplementemic urticarial vasculitis are more likely to show autoantibodies to C1q and vascular endothelial cells.2 The presence of antineutrophilic cytoplasmic antibodies is rare.

The exact frequency is not known in the United States or worldwide.

Previous studies varied in their definitions of the condition. However, when a study in the United Kingdom used consistent criteria restricted to patients diagnosed with vasculitis by biopsy and with urticarial lesions of more than 3 months duration, 2.1% of 1310 patients with urticaria were found to have urticarial vasculitis.

Urticarial vasculitis carries a good prognosis, with most occurrences resolving in months to years. Urticarial vasculitis associated with hypocomplementemia is associated with a greater incidence of coexisting disease (ie, angioedema, connective-tissue disease [primarily SLE], chronic obstructive pulmonary disease). Mortality is rare.

The median age of involvement is 43 years with a range of 15-90 years. While this is primarily a disease of middle-aged adults, it can be seen in persons of any age.

• Patients present with an urticarial eruption, often accompanied by a painful or burning sensation.

o Lesions are generalized wheals or erythematous plaques, occasionally with central clearing, lasting for more than 24 hours in a fixed location (in contrast to urticaria, which resolves in minutes to hours or migrates continually). Petechiae may be noted within the lesions, and they may resolve with ecchymoses or postinflammatory hyperpigmentation.

o Patients may have photosensitivity, lymphadenopathy, arthralgia, angioedema, fever, abdominal pain, dyspnea, and pleural and pericardial effusions.

o Drug induced, such as ACE inhibitors, penicillin, sulfonamides, fluoxetine, and thiazides

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