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dermatomyositis

Friday 4 February 2005

juvenile dermatomyositis

Images

- 4M gottron papules, ulcers, nailfold telangiectasias, heliotrope, prox muscle weakness : juvenile dermatomyositis

- Juvenile dermatomyositis. 15 y/o F w/ Gottron’s lesions, dorsal hands, elbows & knees, ⇈ muscle enzymes & joint problems. Elbow bx

Synopsis

- dermatomyositis-associated cardiomyopathy
- dermatomyositis-associated immune-complex type glomerulonephritis
- dermatomyositis-associated vasculitis

  • dermatomyositis-associated cutaneous arteriolitis
  • chronic endarteropathy
  • digestive ischemic ulceration, digestive perforation
  • systemic angiopathy
  • fatal vascular occlusion
  • autoimmune-mediated vasculopathy

Muscle biopsy

- periodic acid Schiff- (PAS) positive vacuoles
- infiltrates of mononuclear cells
- reduced phosphorylase activity

Biology

- anti-mitochondrial antibody
- mitochondrial anomalies

- perifascicular atrophic fibers
- cytochrome c oxidase (COX)
- succinate dehydrogenase (SDH)

- mitochondrial dysfunction in perifascicular distribution (15614615)
- abnormal SDH and COX histochemical activities in atrophic perifascicular fibers (15614615)