We report a 50-year-old man who presented with a 5-year history of an intermittent widespread pruritic urticarioid rash and fever, fatigue, arthralgia and a monoclonal immunoglobulin-M paraprotein. The patient was initially treated with antihistamines and corticosteroids without the disappearance of symptoms. A skin biopsy from the urticarial rash on the thorax was performed, revealing dermal mononuclear and polymorphonuclear cell infiltrate and normal epidermis. A diagnosis of Schnitzler's syndrome (SS), a rare disorder in which the simultaneous occurrence of monoclonal gammopathy and chronic urticaria is usually observed, was made. After an unsuccessful trial with rituximab at a dosage of 375 mg/sqm weekly for 4 consecutive weeks, the patient was treated with anakinra, an inhibitor of interleukin-la that is thought to be involved in the pathogenesis of the disease, at a dose of 100 mg daily given subcutaneously. He showed a prompt response to the drug and he is still well and symptom-free after 12 months of follow-up. On the basis of both this experience and the review of the literature we conclude that anakinra may be a promising option for the treatment of SS. However, these results need to be confirmed on a larger number of patients.Schnitzler's syndrome (SS) is a rare clinical condition characterized by chronic urticaria and monoclonal IgM gammopathy associated with at least two ofthe following features: intermittent fever, bone pain, arthritis or arthralgia, lymphadenopathy, hepato-or splenomegaly, an acute phase response, abnormal findings in bone morphological investigations (1). To date, about 100 patients with SS have been reported but it is thought that this figure is probably underestimated.Although several hypothesis have been proposed, the pathophysiology ofSS is still unknown. Moreover, treatment of this disorder is difficult and there exists no therapy of choice for SS to date. Several attempts have been made with corticosteroids and immunosuppressive drugs, including thalidomide and rituximab. Recently, anecdotal reports have been published on the successful use of anakinra, an inhibitor of interleukin 1a (lL-1 a). In this regards, we report here on a case of a male patient diagnosed with SS and successfully treated with anakinra.
Case reportIn May 2007, a 50-year-old man presented with a 5-year history of an intermittent widespread pruritic urticarioid rash and fever greater than 38°C, 0394-6320 (2010)