Schnitzler's syndrome with IgG kappa gammopathy

J Dermatol. 2002 Nov;29(11):735-8. doi: 10.1111/j.1346-8138.2002.tb00212.x.

Abstract

A seventy-year-old man with a variant type of Schnitzler's syndrome is reported. Physical examination showed pruritic urticarial lesions on the extremities, arthralgia of knee joints, and intermittent fever. Laboratory investigations revealed a high level of IgG, an increased enythrocyte sedimentation rate, urinary Bence-Jones protein, and an M-bow in serum protein electrophoresis, which was shown to be a monoclonal IgG kappa type. Histological examination showed perivascular neutrophil and lymphocytic infiltration into the upper dermis and diffuse neutrophilic infiltration in the middle dermis. One of the clinical features of typical Schnitzler's syndrome is IgM macroglobulinemia, and this is a very rare case of this syndrome with IgG gammopathy.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aged
  • Biopsy, Needle
  • Follow-Up Studies
  • Humans
  • Hypergammaglobulinemia / complications
  • Hypergammaglobulinemia / diagnosis*
  • Hypergammaglobulinemia / drug therapy
  • Immunoglobulin G / analysis*
  • Immunoglobulin kappa-Chains / analysis*
  • Immunohistochemistry
  • Male
  • Prednisone / therapeutic use
  • Risk Assessment
  • Schnitzler Syndrome / complications
  • Schnitzler Syndrome / drug therapy
  • Schnitzler Syndrome / pathology*
  • Treatment Outcome

Substances

  • Immunoglobulin G
  • Immunoglobulin kappa-Chains
  • Prednisone