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[Schnitzler's syndrome]

A Oborilová1, Z Adam

  • 1II. interní klinika FNsP, Brno-Bohunice.

Vnitrni Lekarstvi
|September 28, 1998
PubMed
Summary
This summary is machine-generated.

Schnitzler syndrome, a rare condition causing urticaria and bone pain, was treated with prednisone and pamidronate. Pamidronate effectively managed bone pain, while prednisone provided temporary relief for skin manifestations.

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Area of Science:

  • Immunology
  • Dermatology
  • Rheumatology

Background:

  • Schnitzler syndrome is a rare autoinflammatory disorder characterized by the co-occurrence of monoclonal immunoglobulin IgM, urticarial rash, and bone abnormalities.
  • This case highlights the diagnostic challenges and therapeutic considerations in managing Schnitzler syndrome.

Observation:

  • A patient presented with monoclonal immunoglobulin IgM, painful osteolysis and osteosclerosis of pelvic bones, and urticarial morphoeae.
  • Urticarial symptoms showed only temporary improvement with corticosteroids and 2-chlorodeoxyadenosine, a treatment for Waldenström's macroglobulinemia.

Findings:

  • Schnitzler syndrome was diagnosed based on the constellation of symptoms.
  • Daily prednisone was required for persistent urticaria, while pamidronate demonstrated efficacy in alleviating bone pain associated with osteolysis and osteosclerosis.

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Implications:

  • This case underscores the importance of recognizing Schnitzler syndrome as a differential diagnosis for chronic urticaria and bone pain.
  • Bisphosphonates, such as pamidronate, represent a viable therapeutic option for managing bone pain in Schnitzler syndrome.