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Benign Hyperglobulinemic PurpuraRelation to Mikulicz's Disease, Sicca Syndrome, and Epidermolysis Bullosa Dystrophica
ROBERT W. GOLTZ, M.D.;
ROBERT A. GOOD, M.D., Ph.D.
Arch Dermatol. 1961;83(1):26-39.
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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 -globulins. Not unexpectedly, the erythrocyte sedimentation rate is also markedly increased. The bleeding and clotting mechanisms are
. . . [Full Text PDF of this Article]
Author Affiliations
MINNEAPOLIS
From the Division of Dermatology, (Francis W. Lynch, M.D., Director), and the Pediatric Research Laboratories, Variety Club Heart Hospital, University of Minnesota (Dr. Goltz, Clinical Associate Professor of Dermatology; Dr. Good, American Legion Memorial Heart Research Professor of Pediatrics).
Footnotes
Submitted for publication July 12, 1960.
Presented in part as an Inaugural Thesis before the 80th Annual Meeting of the American Dermatological Association, Inc., Boca Raton, Fla., April 8-12, 1960.
Aided by grants from the United States Public Health Service, Minnesota Heart Association, and the Minnesota Chapter of the Arthritis and Rheumatism Foundation.
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