Dermatol. praxi. 2011;5(1):28-31

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

prof.MUDr.Zdeněk Adam, CSc.¹, MUDr.Luděk Pour, Ph.D.¹, MUDr.Marta Krejčí, Ph.D.¹, MUDr.Jiří Neubauer², MUDr.Jiří Prášek, CSc.³, prof.MUDr.Roman Hájek, CSc.¹

¹ Interní hematoonkologická klinika LF MU a FN Brno

² Radiologická klinika LF MU a FN Brno

³ Klinika nukleární medicíny LF MU a FN Brno

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

Published: March 17, 2011  Show citation