Cutaneous Manifestations of Primary Sjögren's Syndrome: a Reflection of Vasculitis and Association with Anti-Ro(SSA) Antibodies

Twenty-two symptomatic primary Sjögren's syndrome patients with clinical and histologic evidence of skin disease were studied. Purpura and urticaria were the most frequent clinical cutaneous manifestations. Most of these lesions were associated with a leukocytoclastic angiitis and, less commonly, a mononuclear (lymphocytic) vasculitis. Two main cutaneous syndromes were identified whose clinical, serologic, and histopathologic features were indistinguishable from Waldenström's benign hyperglobulinemic purpura and hypocomplementemic urticaria-like vasculitis. Eighty-four percent of primary Sjögren's syndrome patients with vasculitis demonstrated anti-Ro(SSA) antibodies. Thus, purpura and urticaria may reflect cutaneous vasculitis occurring in the clinical setting of Sjögren's syndrome.