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Incomplete Schnitzler Syndrome.

Mattias A S Henning1, Gregor B E Jemec, Kristina S Ibler

  • 1Mattias A.S. Henning, MD, Department of Dermatology, Zealand University Hospital, Sygehusvej 5, 4000 Roskilde, Denmark; maahe@regionsjaelland.dk.

Acta Dermatovenerologica Croatica : ADC
|July 12, 2020
PubMed
Summary

Schnitzler syndrome (SS) is a rare autoinflammatory condition. A case report shows a patient with clinical SS but without monoclonal immunoglobulin responded well to anakinra, suggesting an incomplete form of the disease.

Area of Science:

  • Immunology
  • Rheumatology
  • Dermatology

Background:

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  • Schnitzler syndrome (SS) is a rare autoinflammatory disorder characterized by chronic urticaria and monoclonal immunoglobulin (Ig) M or G.
  • Clinical features include fever, bone abnormalities, and neutrophilic skin infiltrates, often refractory to standard treatments.
  • Diagnosis typically requires the presence of both urticaria and monoclonal Ig.