Schnitzler syndrome

Abstract

A 63-year-old man presented with non-pruritic urticaria for 14 months,fever of unknown origin for nearly 12 months,and arthralgias of both lower limbs for 10 months.The fever,which was unrelated to the occurrence of skin lesions,appeared once a week on average,and might be as high as 39 ℃.Physical examination revealed irregular fever and lymphadenopathy in both inguinal and axillary regions.Skin lesions,consisting of rosecolored,slightly elevated plaques and papules,affected the trunk,both upper extremities and thighs.Skin biopsy showed perivascular infiltration of neutrophils and lymphocytes in the superficial dermal layer.Laboratory examination exhibited leukocytosis,elevated erythrocyte sedimentation rate and increased C-reactive protein level.Serum protein electrophoresis revealed an IgM-κ component in the γ-globulin zone.Radiographic examination suggested slight hepatosplenomegaly and hepatic cysts.Positron emission tomography/computed tomography (PET-CT) revealed bilateral knee joint effusion,degeneration of cervical vertebrae and lumbar vertebrae,as well as lymphadenectasis in both axillary,inguinal and popliteal regions as well as around external iliac vessels.Wholebody bone scan showed abnormalities of bilateral shoulder joints,elbow joints and knee joints,which were suggestive of arthritis.A diagnosis of Schnitzler syndrome was made.The patient was given prednisone 30 mg per day,and body temperature returned to normal with the improvement of condition on the second day.Subsequently,glucoside tripterygium total 20 mg thrice daily was additionally given,and the dose of prednisone was gradually decreased to 15 mg per day,with a satisfactory control of condition,disappearance of fever,skin eruptions or other discomfort,and marked relief of ostalgia and arthralgia. Key words: Schnitzler syndrome; Urticaria; Diagnosis, differential