Primary benign hypergammaglobulinemic purpura of Waldenstrom masquerading as disseminated Schamberg's purpura.

摘要

Hypergammaglobulinemic purpura of Waldenstrom (HGPW) is characterized by hypergammaglobulinemia, recurring purpura particularly on lower extremities, leukopenia, anemia, an elevated erythrocyte sedimentation rate (ESR), and the presence of rheumatoid factor (RF) indicative of circulating immune complexes. HGPW can be primary or secondary. It is considered primary when there is no other associated disease. Secondary HGPW has a significant association with autoimmune diseases, especially Sjogrens syndrome and lupus erythematosus. We report a case of primary benign HGPW, which presented with recurrent purpuric rash on extremities for the last 10 years and had undergone repeatedly extensive investigations for the same without conclusion.