Schnitzler's syndrome: A female elderly case presenting intractable non-pruritic febrile urticarial rush

Asian Pac J Allergy Immunol. 2020 Mar;38(1):64-66. doi: 10.12932/AP-150818-0394.

Abstract

Schnitzler's syndrome is an acquired autoinflammatory disease characterized by chronic urticarial rash and monoclonal gammopathy (predominantly IgM type). A 75-year-old Japanese woman complained of high fever and non-pruritic urticarial rash appearing almost every day for 3 years. Her abnormal laboratory data included leukocytosis and neutrophilia with elevated erythrocyte sedimentation rate and C-reactive protein level. Hyperglobulinemia of IgA and IgM was also noted. Histological analysis revealed perivascular and interstitial neutrophilic infiltration without any signs of vasculitis. Immunofixation analysis confirmed IgM-kappa-type monoclonal gammopathy. Oral prednisolone initially improved her symptoms, but recurrence was observed upon its tapering. The addition of colchicine successfully controlled her symptoms and allowed a reduction in the dose of systemic steroid.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Colchicine / therapeutic use
  • Female
  • Humans
  • Immunoglobulin M
  • Japan
  • Paraproteinemias / etiology
  • Prednisolone / therapeutic use
  • Schnitzler Syndrome / diagnosis*
  • Schnitzler Syndrome / drug therapy
  • Urticaria / etiology*

Substances

  • Immunoglobulin M
  • Prednisolone
  • Colchicine