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作者等在6例病期长的泛发性硬肿病患者中发现3例伴单细胞系丙种球蛋白病.这3例患者中,男性2例,女性1例;年龄分别为58、65及34岁;病期1年~3年半.皮肤最先发病部位分别为面部、颈部、肩及躯干.患者皆无糖尿病或呼吸道感染史;1例有吞咽困难.体格检查:面颈部及躯体上半部分皮肤均呈木板样硬变,皱襞处皮肤不能捏起;面部无表情,1例且有双唇发硬;舌及舌系带正常;2例在两臂上内侧,其中1例尚在肩部,出现横条状凹痕.化验检查:血沉、血红蛋白、红、白细胞计数、白细胞分类、血清转氨酶、乳酸脱氢酶、醛缩酶及硷性磷酸酶、抗“O”均正常;CPK仅1例稍升高(50u/ml);RA均阴性.血清免疫球蛋白:IgA、IgM、IgE(仅作1例)、C3c及C4均正常.血清蛋白电泳:2例显示额外梯度变化曲线(分别为5.6g/
The authors found in 3 of 6 patients with prolonged eosinophilic encephalopathy were single cell line gammopathy, 3 of which were male and 2 female, with a mean of 58 and 65 34 years of age; duration of 1 to 3 years and a half. The first skin lesions were the face, neck, shoulders and torso patients with no history of diabetes or respiratory tract infection; 1 case of dysphagia. Physical examination: face and neck and The upper half of the body showed a hardwood like skin changes, wrinkles at the skin can not be picked up; facial expression, 1 case and hard lips; tongue and tongue with normal; 2 cases in the medial, in which 1 case Laboratory tests: ESR, hemoglobin, red, white blood cell count, white blood cell classification, serum aminotransferase, lactate dehydrogenase, aldolase and alkaline phosphatase, anti- “O” All were normal; CPK was only slightly elevated in one case (50u / ml); RA was negative.Serum immunoglobulin: IgA, IgM, IgE (only 1 case), C3c and C4 were normal.Serum protein electrophoresis: 2 cases showed Extra gradient curve (5.6 g /