Chronic Urticarial Vasculitis and Immunoglobulin-G Monoclonal Gammopathy: Variant Schnitzler Syndrome

Shilpa Bhartia,1 *Sujoy Khan2

1. Department of Hematology and Hemato-oncology, Apollo Gleneagles Hospital Kolkata, Kolkata, India
2. Department of Allergy and Immunology, Apollo Gleneagles Hospital Kolkata, Kolkata, India
*Correspondence to

Disclosure: The authors have declared no conflicts of interest.
Received: 23.06.17 Accepted: 20.09.17
Citation: EMJ Dermatol. 2017;5[1]:106-112.


Schnitzler syndrome is a rare acquired autoinflammatory disorder that is characterised by recurrent fevers, bone or joint pains, urticarial rash, and monoclonal immunoglobulin M paraprotein, while the variant form has immunoglobulin G monoclonal paraprotein. The cytokine that appears to cause the inflammatory episodes is interleukin-1β, and blocking this cytokine ameliorates almost all symptoms of this disorder. Physicians should be aware of this disorder so that they can recognise this difficult form of urticarial vasculitis and prevent the complication of amyloidosis.

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