[Schnitzler syndrome].
A 60-year-old man has been suffering from worsening chronic urticaria for 15 years. In recent years the skin changes have been associated with bone pain, intermittent fever and pruritus. X-rays show localized bone condensation. Immunoelectrophoretic analysis documents an IgM kappa monoclonal gammopathy. Bone biopsy and bone marrow aspirate revealed no atypical lymphoid infiltration. This syndrome was first described by Schnitzler et al. Other cases have been reported, most of them by French authors. The pathogenesis of the syndrome remains unclear although anti-interleukin-1-alpha-antibodies have been found in several cases. Symptomatic treatment with ibuprofen seems promising.
Lautenschlager
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Dermatologische Universitätsklinik Basel.
Itin
P H
PH
D001168:Arthritis / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D001323:Autoantibodies / Q000032:analysis; D001706:Biopsy; D001853:Bone Marrow / Q000473:pathology; D005335:Fever of Unknown Origin / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D006801:Humans; D007052:Ibuprofen / Q000627:therapeutic use; D007070:Immunoglobulin A / Q000032:analysis; D007074:Immunoglobulin G / Q000032:analysis; D007075:Immunoglobulin M / Q000032:analysis*; D007145:Immunoglobulin kappa-Chains / Q000032:analysis; D007375:Interleukin-1 / Q000276:immunology; D008297:Male; D008875:Middle Aged; D009131:Muscle, Smooth, Vascular / Q000473:pathology; D010265:Paraproteinemias / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D012867:Skin / Q000098:blood supply / Q000473:pathology; D013577:Syndrome; D014581:Urticaria / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D008258:Waldenstrom Macroglobulinemia / Q000188:drug therapy / Q000209:etiology / Q000276:immunology
D001323:Autoantibodies; D007070:Immunoglobulin A; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D007145:Immunoglobulin kappa-Chains; D007375:Interleukin-1; D007052:Ibuprofen
Das Schnitzler-Syndrom.