SCHNITZLERS SYNDROME AS CAUSE OF RECURREN T FEVER OF UNKNOWN ORIGIN

History and clinical findings: For 4 years a 56-year-old woman had bee n suffering from chronic urticaria, In the past two years she had deve loped recurrent fever, each 1-3 days in duration. In the last 6 to 8 m onths she also had severe aching in the legs. There were no significan t findings on physical examination other than non-itching urticarial r ash over trunk and limbs. Investigations: Blood sedimentation rate was increased (maximum 88/110 mm), as were WBC count (16,200/mu l, 83% ne utrophils) and the activity of alkaline leucocyte phosphatase (225 U/l ). Monoclonal IgM gammopathy type kappa and circulating IgM immune com plex (15.4 mg/dl) were demonstrated in serum. Skin biopsy of a new urt icarial lesion revealed vasculitis. Iliac crest biopsy was unremarkabl e and showed no lymphoid cell infiltration. Bone scintigraphy revealed bilaterally increased storage in femor and tibia. Magnetic resonance imaging demonstrated marrow infiltration without space-occupying featu res in the affected femur and tibia. Treatment and course: Conjunction of fever, generalised urticaria, joint/bone pain and monoclonal gammo pathy of kappa type having established the diagnosis of Schnitzler's s yndrome, treatment with ibuprofen was started (initially 1,200 mg dail y by mouth, followed by reduction to 600 mg daily). All symptoms rapid ly improved and the bone changes regressed, but not the monoclonal gam mopathy. But when the ibuprofen dosage had been decreased to 600 mg da ily, the urticaria recurred, though in milder form. Conclusion: This c ase demonstrates the efficacy of ibuprofen in the treatment of the Sch nitzler's syndrome. Furthermore reversibility of scintigraphic bone le sions could be demonstrated under ibuprofen treatment.