Abstract
IntroductionSchnitzler Syndrome is an uncommon, inflammatory condition that presents with a constellation of chronic unremitting urticaria, fever, bone pain, arthralgia or arthritis, and a monoclonal IgM gammopathy. There is usually neutrophilia and raised inflammatory markers. Delayed diagnosis is common and treatment often unsuccessful.Case presentationWe report the case of a 43-year-old caucasian man who presented with urticaria unresponsive to conventional therapy. There was considerable delay in recognition of this as Schnitzler Syndrome, and symptoms were unresponsive to conventional immunosuppressive therapy.Commencement of anakinra was associated with a rapid and sustained clinical response.ConclusionSchnitzler Syndrome is a rare disorder that mimics chronic idiopathic urticaria. This diagnosis should be considered in patients with urticaria unresponsive to antihistamines and conventional immunosuppressive therapy. Anakinra is an effective treatment although further studies are required, to determine long term therapeutic requirements and assess any potential adverse effects.
Highlights
Schnitzler Syndrome is an uncommon, inflammatory condition that presents with a constellation of chronic unremitting urticaria, fever, bone pain, arthralgia or arthritis, and a monoclonal IgM gammopathy
Schnitzler Syndrome is a rare disorder that mimics chronic idiopathic urticaria. This diagnosis should be considered in patients with urticaria unresponsive to antihistamines and conventional immunosuppressive therapy
Chronic idiopathic urticaria (CIU) is a common, benign condition for which patients are frequently referred to allergy or dermatology clinics
Summary
SS is a rare cause of a common symptom (urticaria), but this typically occurs in patients with other systemic symptoms. These systemic symptoms show considerable overlap with AOSD and immunologists, dermatologists and rheumatologists all need to be aware of this potential diagnosis. Patients with SS have a significant risk of late malignancy and should be carefully monitored for its development. Http://www.casesjournal.com/content/1/1/348 ever the introduction of anakinra has been very effective in this and 4 other published cases. Our experience suggests that treatment with anakinra is likely to be required in the long term in order to control symptoms, reduce inflammation and diminish the risk of long term side effects
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