Perifascicular atrophic fibers in childhood dermatomyositis with particular reference to mitochondrial changes.

Ten of 17 muscle biopsy specimens from 15 patients with childhood dermatomyositis (DM) showed distinct perifascicular atrophy. The atrophic fibers showed the following characteristics: (1) decreased cytochrome c oxidase (CCO) activity, (2) rare positive reaction with acridine orange (AO) staining, (3) type 2C reaction in 7.9% (0.4-17.5%) of the fibers, (4) an increased number of activated satellite cells, (5) mitochondria which were increased in number but decreased in size, (6) a significantly decreased CCO activity in isolated mitochondria (51.6 +/- 30.3 nmol/min per mg mitochondrial protein) as compared with that in the controls (103.6 +/- 41.5). The major pathogenetic mechanism in muscles in childhood DM is thought to be ischemia due to involvement of the microvasculature. The presence of type 2C fibers and increased numbers of activated satellite cells reflect a focal repair process taking place concomitantly in the damaged myofibers. Mitochondrial enzyme defect, especially CCO deficiency is present not only in genetic disorders with mitochondrial involvement but in other neuromuscular disorders including inflammatory myopathies.