Urticarial Vasculitis Syndrome

The usual clinical presentation of necrotizing vasculitis is a palpable purpura; other possible features are macules, papules, pustules, ulcers, or scars. A few decades ago, attention was called to the fact that urticaria can also be a manifestation of cutaneous vasculitis (McCoombs 1965). The clinical aspects and underlying pathomechanisms of urticarial vasculitis were studied two decades later (McDuffie et al. 1973, Sissons et al. 1974, Soter et al. 1974, Agnello et al. 1975 and 1976), and by the early eighties, more than 100 patients with the disease had been reported in the literature (Sanchez et al. 1982, Wanderer et al. 1983).