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局限性硬皮病可合并肌病,但对其骨骼肌的组织病理学很少研究.作者认为这种病的致病原因可能为自身免疫性胶原性血管病的作用.患者女性,4岁.右手第一、二指出现硬化斑,并向右上肢内侧逐渐扩展.5岁时检查发现:(1)右上肢内侧可见明显肉色硬化斑;(2)右上肢与肩胛带肌肉萎缩,特别是肱二头肌、肱桡肌和拇指短肌;(3)腕和肘关节屈曲性挛缩,右上肢近端和远端肌肉中度无力,X线照相显示右侧桡骨萎缩.抗核抗体滴定度1∶2560,抗DNA抗体滴定度1∶2560,红斑狼疮血凝试验阳性.其它有关检查未见异常.
The limitations of scleroderma may be combined with myopathy, but its histopathology of skeletal muscle rarely studied, the author believes that the pathogenesis of this disease may be the role of autoimmune collagen vascular disease. Female patients, 4 years old. The first and second fingers of the right hand showed sclerosis and gradually extended to the inside of the right upper extremity. Examination at age 5 revealed that: (1) a marked flesh-shaped sclerosis was visible on the inside of the right upper extremity; (2) muscle atrophy of the right upper limb and the shoulder girdle, Biceps, brachioradialis and thumb short muscles; (3) wrist and elbow flexion contractures, right and left upper extremity muscle weakness in the proximal and distal, radiographs show the right radial atrophy. Anti-nuclear antibody titer 1 : 2560, anti-DNA antibody titer 1:2560, positive test for lupus erythematosus coagulation test. Other related tests showed no abnormalities.