Schnitzler syndrome: a rare etiology of systemic urticaria

Introduction. - The Schnitzler's syndrome first described in 1972, associat es urticaria, bone pain, and monoclonal IgM gammapathy. Exegesis. - A 50-year-old man presented symptoms of urticaria restricted to the trunk and lower members, with episodes of fever accompanied by inflamm atory pain in the knees and legs. Slight deterioration of his general condi tion was also observed. Biological findings showed the existence of an infl ammatory syndrome. Electrophoresis and immunoelectrophoresis provided evide nce for the existence of underlying IgM gammapathy. Bone X-ray demonstrated the presence of tibial and peroneal metaphysis thickening, with hyperfixat ion on bone scintigraphy The patient's condition improved after cortisone a nd colchicine treatment, allowing decrease in coricosteroid doses. Two year s later, except for urticaria, clinical features have disappeared and no he matological disorder has been observed. Conclusion. - This patient presented Schnitzler's syndrome with unusual bon e involvement including peri-articular pain and periosteum affixing of the tibias. Therapy with cortisone and colchicine proved to be efficacious, whi ch is also rare in this disease. The syndrome outcome is unforeseeable, oft en benign. As in the princeps case, hematologic disorders can evolve to lym phocytoma or myeloma, and call for regular follow-up of the patients due to potential occurrence of hematological disease. (C) 2000 Editions scientifi ques et medicales Elsevier SAS.