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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)