Sjögren's syndrome. Association of cutaneous vasculitis with central nervous system disease

We describe a group of patients with Sjögren's syndrome, who commonly present to dermatologists with cutaneous manifestations of vasculitis. Two specific clinically recognizable forms of cutaneous vasculitis predominate: palpable purpura of the lower extremities (Waldenström's benign hypergammaglobulinemic purpura) and urticarialike vasculitis. Two pathologic types of cutaneous vasculopathy are demonstrated, one leukocytoclastic and the other mononuclear. The leukocytoclastic vasculopathy is associated with high titers of Ro(SS-A) and La(SS-B) autoantibodies (detected by gel double-diffusion techniques) and general serohyperreactivity. In marked contrast, the mononuclear vasculopathy is associated with low titers of Ro(SS-A) and La(SS-B) autoantibodies (detected by enzyme-linked immunosorbent assay but not gel double-diffusion techniques) and general serohyporeactivity. Approximately 70% of patients with Sjögren's syndrome and cutaneous vasculitis have also developed peripheral and/or central nervous system disease. The pathogenesis of the nervous system disease is unknown, but preliminary data suggest a vasculopathy.