Benign Hyperglobulinemic Purpura

Abstract
This paper deals with the relationships between benign hyperglobulinemic purpura, the sicca syndrome of Gougerot and Sjögren, and Mikulicz's disease. Patients demonstrating these relationships will be described, and a hitherto unreported association of hyperglobulinemic purpura and epidermolysis bullosa dystrophica proposed. No complete review of the literature on these subjects will be attempted since recent excellent reviews are readily available.1,2 Briefly, benign hyperglobulinemic purpura of Waldenström has been reported in about 30 patients. Purpura appears on the lower legs, usually after prolonged standing or exercise, although one patient had areas of extensive cutaneous hemorrhage involving the trunk and upper extremities as well as the legs.3 A crucial feature is the presence of an unexplained dysproteinemia. The serum proteins are elevated, sometimes markedly, the elevation being confined to the globulin fractions, especially the γ-globulins. Not unexpectedly, the erythrocyte sedimentation rate is also markedly increased. The bleeding and clotting mechanisms are