[Systemic urticaria, sclerosing osteopathy, monoclonal gammopathy (Schnitzler syndrome). Apropos of a case].
A 65 years old man has been suffering from the upper limbs and legs for 2 years. Bone pain was associated with a non pruritic chronic urticaria and fever. Bone scintigraphy showed lesions of the lower limbs. X rays showed only one sclerotic bone lesion. Immunoelectrophoretic analysis showed an IgM kappa monoclonal gammapathy. Bone marrow aspirate and bone marrow biopsy were normal. The association of chronic urticaria, bone pain and macroglobulinemia has been first reported by L. Schnitzler. Seventeen other cases have been reported in the literature. The pathogenesis of the syndrome is unknown although anti-interleukin 1 alpha autoantibodies were found in several cases (8 out of 13).
De Saint-Pierre
V
V
Service de Rhumatologie, CHU Brest.
Ehrhart
A
A
Baron
D
D
Plantin
P
P
Le Goff
P
P
D000368:Aged; D001842:Bone and Bones / Q000473:pathology*; D006801:Humans; D008297:Male; D010265:Paraproteinemias / Q000150:complications*; D012598:Sclerosis; D013577:Syndrome; D014581:Urticaria / Q000150:complications*
Urticaire systémique, ostéopathie condensante, gammapathie monoclonale (syndrome de Schnitzler). A propos d'un cas.