Dermatológia pre prax 2/2011

Schnitzler syndrome – chronic urticaria + monoclonal gammopathy IgM + osteolytic-osteosklerotic bone changes

Schnitzler syndrome is a rare disorder characterized by chronic urticaria, the presence of monoclonal class IgM immunoglobulin, lymphadenopathy and fevers. We report on a patient who was diagnosed in 1996 and over the next 12 years underwent a number of therapies. Bone pain associated with proven osteolytic and osteosclerotic lesions resolved on regular bisphosphonate treatment with pamidronate and, later, clodronate. The patient had transient benefit from treatments with interferon-alpha and with PUVA. No therapeutic effects were seen with high-dose dexamethasone, 2-chlordeoxyadenosin, cyclosporine, or the combination of bortezomib, thalidomide, and dexamethasone. However, on therapy with anakinra the patient’s skin symptoms resolved completely and the values of CRP and hemoglobin normalized.

Keywords: Schnitzler syndrome, gammapathy, urticaria, anakinra