SCHNITZLERS SYNDROME - REPORT OF A NEW CASE AND A REVIEW OF THE LITERATURE

Schnitzler syndrome is a rare condition characterized by chronic non-p ruritic urticaria, recurrent fever, bone pain, osteocondensation, and monoclonal IgM gammopathy without features of lymphoproliferative dise ase. We describe the case of a 44-year-old man with an 8-year history of bone pain with hyperostosis and a 5-year history of chronic non-pru ritic urticaria, associated with fever, hyperleukocytosis, hepatomegal y: serum monoclonal IgM-kappa. Systemic treatment with steroids was ef fective against bone pain but was ineffective in controlling the urtic aria. We also review 35 cases. No adequate treatment has yet been foun d. The pathogenesis is unclear and the role of the IgM component in th e induction of urticaria has nor been established.