ABSTRACT
Episodes of recurrent urticaria are an uncommon manifestation of cutaneous necrotizing vasculitis that involves venules. Some patients have a multisystem disorder with extracutaneous manifestations. Although the variety of skin lesions and extracutaneous manifestations have led to a plethora of diagnostic appellations (1-18), the terms urticarial vasculitis or venulitis currently are widely used. A subset of patients with urticarial venulitis and systemic manifestations, hypocomplementemia with low Clq levels, and an anti-Clq autoantibody has been designated as having the hypocomplementemic-urticarial-vasculitis syndrome (HUVS) (17). Urticarial venulitis may occur in association with a variety of medical disorders; however, it may be an idiopathic condition (Table I).
