Hypergammaglobulinemic purpura, Sjögren's syndrome

Reference work entry
DOI: https://doi.org/10.1007/3-540-29662-X_1286

Synonyms

Hypergammaglobulinemic purpura of Waldenström (HGPW), benign hypergammaglobulinemic purpura of Waldenström.

Definition

Hypergammaglobulinemic purpura may occur in patients with Sjögren's syndrome.

This is manifest as an assymetrical, non-thrombocytopenic purpura of the lower extremities. It has also been described with systemic lupus erythematosis and hepatitis C. Other clinical features may include constitutional manifestations, elevated acute phase reactants such as erythrocyte sedimentation rate, polyclonal hypergammaglobulinemia, rheumatoid factor, and anti-SSA/SSB antibodies. Histologically, leukocytoclastic vasculitis has been detected in some patients.

Therapy

Pharmacological

The condition does not require aggressive therapy. Treatment with prednisolone has been reported to improve skin and hematologic manifestations. Other agents used include non-steroidal anti-inflammatory drugs, hydroxychloroquine, gold salts, and cyclophosphamide.

General Therapeutic Measures

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References

  1. Katayama I (1995) Clinical analysis of recurrent hypergammaglobulinemic purpura associated with Sjögren's syndrome. J Dermatol 22(3):186–90PubMedGoogle Scholar
  2. Wada M, Kamimoto H, Park SY, et al (2001) Autoimmune hepatitis concomitant with hypergammaglobulinemic purpura, immune thrombocytopenia, and Sjögren's syndrome. Intern Med 40(4):308–11PubMedGoogle Scholar
  3. Yamamoto T Yokoyama (1997) Hypergammaglobulinemic purpura associated with Sjögren's syndrome and chronic C type hepatitis. A J Dermatol 24(1):7–11Google Scholar

Copyright information

© Springer-Verlag 2004