Cutaneous leukocytoclastic vasculitis with hyperglobulinemia and splenomegaly. A variant of hyperglobulinemic purpura of Waldenström.
Patient with hepatosplenomegaly and recurrent small purpuric lesions of cutaneous leukocytoclastic vasculitis was found to have a polyclonal elevation of gamma-globulin and a persistently elevated ESR. Circulating immune complexes were detected that support the concept of a postulated immune complex pathogenesis. Indomethacin therapy was ineffective, but prednisone therapy resulted in notable clinical improvement and the return of elevated immunoglobulin levels to the normal range. Patients with hyperglobulinemic purpura, which may be a subset of leukocytoclastic vasculitis, should be examined for associated conditions.
D000328:Adult; D000936:Antigen-Antibody Complex / Q000032:analysis; D001799:Blood Sedimentation; D005260:Female; D006529:Hepatomegaly; D006801:Humans; D009336:Necrosis; D011241:Prednisone / Q000627:therapeutic use; D011694:Purpura, Hyperglobulinemic / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D013163:Splenomegaly; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D005719:gamma-Globulins / Q000032:analysis
D000936:Antigen-Antibody Complex; D005719:gamma-Globulins; D011241:Prednisone