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Portable Thermographic Screening for Detection of Acute Wallenberg's Syndrome
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[Schnitzler syndrome].

C Ilbert1, M Sabatiello2, W Kurth3

  • 1Faculté de Médecine, ULiège, Belgique.

Revue Medicale De Liege
|August 12, 2020
PubMed
Summary
This summary is machine-generated.

Schnitzler syndrome, a rare condition, involves chronic urticarial rash and monoclonal gammopathy. Anakinra, an IL-1 receptor antagonist, effectively treated a patient with this syndrome, resolving skin rash and pain.

Keywords:
AnakinraInterleukinMonoclonal gammopathy1Schnitzler syndrome

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Area of Science:

  • Rheumatology
  • Dermatology
  • Immunology

Background:

  • Schnitzler syndrome is a rare autoinflammatory disorder characterized by the co-occurrence of monoclonal gammopathy and chronic urticarial rash.
  • Diagnosis can be challenging due to overlapping symptoms with other conditions like urticarial vasculitis.

Observation:

  • A 57-year-old woman presented with a decade-long history of urticarial exanthema, monoclonal immunoglobulin M Kappa gammopathy, and arthralgia.
  • Initial treatment with colchicine for presumed urticarial vasculitis was ineffective.

Findings:

  • The patient was diagnosed with Schnitzler syndrome based on the constellation of symptoms and laboratory findings.
  • Treatment with anakinra, an interleukin-1 receptor antagonist, led to remarkable improvement in skin rash, bone pain, and inflammatory markers.

Implications:

  • This case highlights the importance of considering Schnitzler syndrome in patients with unexplained urticarial eruptions and monoclonal gammopathy.
  • Targeting interleukin-1 with anakinra represents a promising therapeutic strategy for Schnitzler syndrome, offering significant symptom relief and improving inflammatory markers.