Tomková H, Shirafuji Y, Arata J
Department of Dermatology, Okayama University Medical School, 2-5-1 Shi-kata-cho, 700 Okayama City, Japan.
Eur J Dermatol. 1998 Mar;8(2):118-21.
We report the case of a 50-year-old man with a 4-year history of high spiking fever accompanied by a widespread, urticarial, non-pruritic or only sometimes mildly pruritic eruption and arthralgia. He also had generalized lymphadenopathy, hepatosplenomegaly, and hyperosteoses of the lower lumbar spine. Laboratory examination revealed an elevated erythrocyte sedimentation rate, elevated white blood cell and platelet counts, hypoalbuminemia, and elevated serum IgM with IgM kappa monoclonal immunoglobulin. We diagnosed his condition as Schnitzler's syndrome, in contrast to the diagnosis of adult onset Still's disease, for which he had been initially followed up by his internist. We compare clinical and histopathological findings for both diseases and, as this patient meets two of the six existing diagnostic criteria for adult onset Still's disease, we propose that Schnitzler's syndrome is an important entity to be added to the list of differential diagnoses for adult onset Still's disease.
我们报告了一例50岁男性患者，其有4年高热病史，伴有广泛的荨麻疹样皮疹，无瘙痒或仅有时轻度瘙痒，以及关节痛。他还出现全身淋巴结肿大、肝脾肿大和下腰椎骨质增生。实验室检查显示红细胞沉降率升高、白细胞和血小板计数升高、低白蛋白血症，以及血清IgM升高伴IgM κ单克隆免疫球蛋白。我们将他的病情诊断为施尼茨勒综合征，与成人斯蒂尔病的诊断形成对比，他最初由内科医生按照成人斯蒂尔病进行随访。我们比较了这两种疾病的临床和组织病理学表现，由于该患者符合成人斯蒂尔病现有六项诊断标准中的两项，我们建议将施尼茨勒综合征作为一个重要的实体添加到成人斯蒂尔病的鉴别诊断列表中。
