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Introduction
Schnitzler syndrome (SS) is a rare and acquired systemic disease with many common features of the group of inherited diseases referred to as auto-inflammatory syndromes, presenting with chronic urticaria and monoclonal gammopathy [1]. Conventional therapies including anti-histamines, anti-inflammatory drugs, steroids and immunosuppressive drugs, are usually ineffective. However, the IL-1 receptor antagonist anakinra was found to rapidly control all the symptoms of this syndrome [1].
Case Report
We report a 50-year-old Caucasian woman with SS for 10 years. The leading symptoms were chronic urticarial rash (nonpruritic) (Fig. 1), recurrent fever up to 39 °C, fatigue, general malaise and bone pain. Laboratory analyzes conducted regularly showed permanent leukocytosis with neutrophilia, elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) and a monoclonal gammopathy IgM kappa. Bone marrow biopsy was never consistent with malignancy. Bone scintigraphy was performed and hyper-fixation was observed on distal left femur, proximal right femur and skull. CT scan revealed hepatomegaly and cervical lymphadenopathy. A lymph node biopsy excluded malignant infiltration. Histologic examination from an urticarial lesion showed a mixed inflammatory infiltrate (predominantly of neutrophils) in the dermis with no evidence of vasculitis (Fig. 2). All these findings were consistent with the diagnosis of SS according to the Lipsker et al. [2] criteria. The disease had a major impact on quality of life of our patient and the manifestations were refractory to treatment with hydroxyzine, prednisolone, hydroxychloroquine, naproxen and colchicine. As the disease was not controlled, the patient was started on anakinra...