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Participant 030


Schnitzler syndrome

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Male, age 70 with Schnitzler syndrome

Date of Report

Aug 22, 2017

Description

Around age 60, the patient started having recurrent episodic fevers, with associated muscle/joint pain and a hive-like rash across his upper chest and abdomen that would come and go with the fevers. He had extensive evaluation for occult infection or malignancy that was unrevealing.  He was trialed on numerous immunosuppressive regimens.   At present, with his diagnosis of Schnitzler syndrome, he is on tocilizumab and prednisone.

Symptoms / Signs
  • Abnormal protein in blood (monoclonal gammopathy)
  • Skin rash (that resembles hives) associated with the fevers
  • Episodic fevers
  • Arthritis/arthralgias
  • Bone pain
  • Enlarged lymph nodes (lymphadenopathy)
  • Enlarged liver or spleen
  • Elevated white blood cell count or elevated inflammatory markers
Current Treatments
  • Tocilizumab (IL-6 receptor antagonist), prednisone
Prior Treatments
  • Colchicine, plaquenil, methotrexate, prednisone, anakinra
Considered treatments
Previously Considered Diagnoses
  • Infection
  • Malignancy
  • Drug reaction
  • Sweet syndrome
  • Still’s disease
  • Cryopyrin-associated autoinflammatory disease
  • Tumor necrosis factor receptor-1 associated periodic syndrome
Other Photographs
Genetic Variants of Interest
Contact

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