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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)