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Schnitzler syndrome
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Schnitzler syndrome

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Contributors: Annie Yang MD, Lowell A. Goldsmith MD, MPH
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Synopsis

Schnitzler syndrome is a rare hematologic disorder with monoclonal gammopathy that manifests with a chronic urticarial rash, intermittent fever, arthralgias, and myalgias. Other manifestations include lymphadenopathy and hepatosplenomegaly. There is slight male predominance, and mean age of onset is 51 years.

There is consensus among experts for use of the Strasbourg criteria, which have a definite and a probable diagnosis category and can direct management.

Various treatments have been tried with variable results. Successful treatment with anakinra, an IL-1 receptor antagonist, has been documented in case reports.

Codes

ICD10CM:
D47.2 – Monoclonal gammopathy

SNOMEDCT:
402415001 – Schnitzler syndrome

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Adult-onset Still disease (AOSD) – Both Schnitzler syndrome and AOSD are characterized by urticarial rash, fever, joint pain, and leukocytosis. Distinguishing features of AOSD include elevated ferritin levels, increased transaminases, and pharyngitis.
  • Rheumatoid arthritis – Positive anti-cyclic citrullinated peptide (anti-CCP) antibodies and rheumatoid factor; elevated inflammatory markers erythrocyte sedimentation rate and C-reactive protein (CRP); synovitis on exam with pain, swelling, and warmth of small joints of the fingers, wrists, and ankles; morning stiffness lasting more than 1 hour that improves with activity.
  • Cryopyrin-associated periodic syndrome – Rare genetic disease involving the cryopyrin protein. Muckle-Wells syndrome presents with fever, hive-like rash, red eyes, joint pain, and severe headaches with vomiting; deafness / age-related hearing loss can develop. Episodes typically last 1-3 days. Familial cold autoinflammatory syndrome is triggered by exposure to cold or other environmental factors; it presents with a hive-like rash, fever, chills, nausea, headaches, and joint pain. Episodes typically last 1 day.
  • Urticarial vasculitis – Presents similarly to Schnitzler syndrome with rash, fever, and joint pain. Decreased levels of complement as a result of increased consumption and anti-C1q antibodies are present in urticarial vasculitis but not in Schnitzler syndrome. Unlike true vasculitides, skin biopsy in Schnitzler syndrome will not show fibrinoid necrosis.
  • Cryoglobulinemic vasculitis – Low complement values and presence of cryoglobulins causing systemic effects such as skin and renal disease; associated with hepatitis C, malignancy, or autoimmune condition; three different types, if polyclonal associated with Meltzer triad of palpable purpura, arthralgias, and myalgias.
  • Systemic lupus erythematosus – SOAP BRAIN MD mnemonic (serositis, oral ulcers, arthritis, photosensitivity, blood disorders, renal involvement, antinuclear antibodies, immunologic phenomena, neurologic disorder, malar rash, discoid rash); must fulfill 4 of 11 of criteria according to the American College of Rheumatology.
  • Chronic idiopathic urticaria
  • Monoclonal gammopathy of undetermined significance (MGUS) – Clinically does not cause signs or symptoms; usually affects the elderly with peak age older than 85 years.

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Therapy

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References

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Last Updated: 07/18/2017
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Schnitzler syndrome
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Schnitzler syndrome : Bone pain, Arthralgia, Recurring fever episodes, Chronic urticarial rash
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