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From the departments of Neurology-Neurosurgery and Pediatrics, McGill University, The Montreal Neurological Institute, and Montreal Children's Hospital, Montreal, Quebec, Canada.
The childhood type of 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 occasionally 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.
Dr. Carpenter's address is Montreal Neurological Institute, 3801 University St., Montreal, Quebec, Canada H3A 2B4.
This study was supported in part by The Medical Research Council of Canada, and The Isaac Walton Killam Memorial Fund of The Montreal Neurological Institute, and The Muscular Dystrophy Association of Canada.
Received for publication July 28, 1975.
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