Carpenter S, Karpati G, Rothman S, Watters G
Neurology. 1976 Oct;26(10):952-62. doi: 10.1212/wnl.26.10.952.
The childhood type fo dermatomyositis, which occurs in children and young adults, shows a specific constellation of pathologic changes in muscle. Capillary necrosis leads to capillary loss, generally starting on the periphery of muscle fascicles. Electron microscopy discloses undulating tubules in endothelial cells, lymphocytes, pericytes, and pseudosatellite cells. The muscle fiber damage is coextensive with capillary damage and probably results from progressive ischemia. The muscle cells, before atrophying, show mitochondrial elongation, Z disk streaming, focal myofibrillary loss, and occassionally selective thick filament loss. Muscle cell necrosis is rare and limited to infarctlike lesions. Inflammatory infiltrates, if present, occur only in connective tissue septa. The cause of the capillary damage has not been determined.
儿童型皮肌炎发生于儿童和青年,其肌肉呈现特定的病理变化组合。毛细血管坏死导致毛细血管缺失,通常始于肌束周边。电子显微镜检查显示内皮细胞、淋巴细胞、周细胞和假卫星细胞中有波浪状小管。肌纤维损伤与毛细血管损伤范围一致,可能由进行性缺血所致。肌肉细胞在萎缩前表现为线粒体延长、Z盘移位、局灶性肌原纤维缺失,偶尔还有选择性粗肌丝缺失。肌肉细胞坏死罕见,仅限于梗死样病变。若有炎症浸润,仅见于结缔组织间隔。毛细血管损伤的原因尚未确定。