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Refractory urticaria and the importance of diagnosing Schnitzler’s syndrome
  1. Tina Nham1,
  2. Carol Saleh1,
  3. Derek Chu1,
  4. Stephanie L Vakaljan1,
  5. Jason A Ohayon1 and
  6. Deborah M Siegal1,2
  1. 1 Department of Medicine, McMaster University, Hamilton, Ontario, Canada
  2. 2 Population Health Research Institute, McMaster University, Hamilton, Ontario, Canada
  1. Correspondence to Dr Deborah M Siegal, siegald{at}


A 52-year-old man presented with chronic urticaria that was refractory to standard chronic spontaneous urticaria (CSU) treatment. Over time, he developed systemic symptoms including fatigue, weight loss, arthralgia and bone pain. His laboratory investigations also became significant for microcytic anaemia, neutrophilia and elevated C reactive protein, erythrocyte sedimentation rate and IgE levels, in addition to an IgM monoclonal protein. He achieved only partial remission with typical medications for CSU including omalizumab, cyclosporine and cetirizine. After 6 years, his worsening symptoms and abnormal investigations led to a rare diagnosis of Schnitzler’s syndrome and a trial of the interleukin 1 receptor antagonist, anakinra, which caused a rapid and complete resolution of his symptoms.

  • immunology
  • biological agents

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  • Contributors TN organised and interpreted the clinical data, created the manuscript figures and wrote the article. CS, DC and DMS participated in the conception and design of the article, interpreted the data and revised the manuscript. SLV participated in the conception of the article, acquired and interpreted the data and revised the manuscript. JAO participated in the conception and design of the article and revised the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Patient consent for publication Obtained.