Schnitzler syndrome: A rare cause of chronic recalcitrant urticaria

Schnitzler’s syndrome is characterized by an unremitting chronic urticarial rash associated with monoclonal immunoglulin M (IgM or rarely IgG) gammopathy that often progresses into systemic symptoms of inflammation such as recurrent fever, muscle, bone and joint pain, and enlarged lymph nodes. Chronic urticaria is the hallmark of this condition and is present in almost all cases, followed by intermittent fevers in 72% of the patients. There is no biological diagnostic marker, which often leads to a delay in diagnosis. Diagnosis is based on the Strasbourg criteria after exclusion of other causes of systemic inflammation including haematological, infectious and auto-immune conditions. Patients with Schnitzler’s syndrome are at a high risk of developing AA amyloidosis and lymphoproliferative disorders, making timely diagnosis crucial. Interlukin 1 (IL-1) inhibition is the most consistent and effective therapy for patients. Anakinra is a recombinant human IL-1 receptor antagonist that reduces the activity of IL-1, which effectively produces prolonged suppression of the disease. We report a case of chronic recalcitrant urticiaria in a female lasting over two years. The presentation was non-specific and mimicked the spectrum of low grade lymphomas or Waldernstrom’s macroglobulinaemia and other systemic inflammatory diseases. The patient was diagnosed with Schnitzler syndrome and a rapid clinical and biochemical response to anakinra (IL-1 receptor antagonist) was noted.

Dr. Surabhi Sharma