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Urticarial vasculitis is a small-vessel vasculitis characterized clinically by urticaria and microscopically by leukocytoclastic vasculitis. Hypocomplementemic urticarial vasculitis syndrome is a rare subtype of urticarial vasculitis with organ involvement and persistent, acquired hypocomplementemia. Pulmonary involvement, usually seen as diffuse alveolar damage, is an uncommon complication and a significant cause of morbidity and mortality. Here we present a rare case of hypocomplementemic urticarial vasculitis syndrome complicated by diffuse alveolar damage and describe the beneficial effect of cyclophosphamide-dexamethasone.
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