Schnitzler syndrome in a patient with a family history of monoclonal gammopathy

Research output: Contribution to journalArticle

Abstract

Schnitzler syndrome is a rare disease characterized by chronic urticaria and a monoclonal gammopathy, most commonly IgM with light chains of the kappa type. There are currently no known risk factors associated with development of the disease. We report a case of Schnitzler syndrome in a 48-year-old man with a family history of monoclonal gammopathies. The patient’s disease has been well controlled with anakinra therapy. Our case may contribute to a better understanding of the etiology of Schnitzler syndrome as his history could suggest a hereditary predisposition for the disease. Further studies are necessary to determine whether a genetic component of Schnitzler syndrome exists, as first-degree relatives of patients with monoclonal gammopathies may be at risk for the development of the disease.

Original languageEnglish (US)
Article number5
JournalDermatology Online Journal
Volume24
Issue number1
StatePublished - Jan 1 2018

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Schnitzler Syndrome
Paraproteinemias
Interleukin 1 Receptor Antagonist Protein
Inborn Genetic Diseases
Urticaria
Rare Diseases
Immunoglobulin M
History
Light

Keywords

  • Monoclonal gammopathy
  • Multiple myeloma
  • Schnitzler syndrome
  • Waldenström macroglobulinemia

ASJC Scopus subject areas

  • Dermatology

Cite this

Schnitzler syndrome in a patient with a family history of monoclonal gammopathy. / Wilmas, Kelly; Aria, Alexander; Torres Cabala, Carlos Antonio; Lu, Huifang; Duvic, Madeleine.

In: Dermatology Online Journal, Vol. 24, No. 1, 5, 01.01.2018.

Research output: Contribution to journalArticle

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