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KMID : 0363219950330010183
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Korean Journal of Dermatology
1995 Volume.33 No. 1 p.183 ~ p.187
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Systemic Scleroderma in Childhood
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박욱화
박태현/박경득/박동식/전수일
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Abstract
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The onset of systemic scleroderma in the first decade of life occurs in less than 1.5% of instances. Systemic scleroderma in childhood have been characterized by less severe visceral involvement and a benign course. We report a boy, aged 7 years, with a history of Raynaud's phenomenon and dysphagia. He had a mask-like face, flexion contracture of hands and knee joints, digital pitting scars, ichthyosiform skin change on legs and hyperpigmented induration on all extremities and trunk. Antinuclear antibodies were positive in nucleolar type and Scl-70 antibody was also present. On radiologic study, the pattern of reflux esophagitis and resorption of the distal ends of all phallanges were noted. Histopathological findings were compatible with scleroderma. He was treated with Penicillamine-d and oral corticosteroids with good improvement.
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KEYWORD
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