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The childhood dermatomyositis is much less frequently seen than in the adult, and infantile form is more rare. It differs in many respects from the adult form of the disease. One of them is pathological features primarily involved in the small intramuscular blood vessels. This is a presentation of the infantile form exceptionally dystrophic dominant pattern in muscle biopsy.<BR>The patient is a 1-year-l-month-old girl who had been in good health and development until 9 months of age, when mother noted dusky erythematous discoloration of face and limbs. They were getting worse to skin ulcerations which fairly responded to topical corticosteroid. Because of gradual weakness, regression of motor development, anorexia, low grade fever and relapsing skin lesions, she was admitted to the SLH.<BR>Physical examinations revealed heliotrope eyelids, dusky erythematous or hyperpigmented skin with ulcers in the face, limbs and trunks. Muscle weakness is generalized, more involved in the proximal muscles so that she could not crawl and sit up alone. Lab shows; ESR 25 mm/hr, CRP-negative, GOT 118 u, GPT 36 u, LDH 1, 270 u, ALD 19 u, CPK 1, 104 u, Muscle biopsy shows minimum infiltration of mononuclear cells to the intramuscular vessels and moderate to severe dystrophic changes of muscle fibers.<BR>Three weeks treatment with 2 mg/kg of oral prednisolone is dramatic with rapid improvement of muscle weakness, muscle enzymes and skin manifestations. But skin manifestations are relapsing on tapering prednisolone.<BR>Characteristic vasculitis in the muscle biopsy of childhood dermatomyositis is rather scarce in our case. Instead, dystrophic changes are main features. Whether these findings are due to immunological immaturations or different mechanisms is unknown, yet the possibility of the direct attack to muscle fiber itself or membrane should be in mind as a pathogenesis of the illness.
- 一般社団法人 日本小児神経学会の論文
一般社団法人 日本小児神経学会 | 論文
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