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要約 背景:限局性強皮症は原因不明の限局性の皮膚結合織の硬化性病変で5型に分類され,顔面や頭部に生じる場合は剣創状強皮症という。目的:剣創状強皮症に併発したぶどう膜炎の女児の報告。症例:4歳3か月の女児が健康診断で発見された左眼視力不良で受診した。1年前から剣創状強皮症で皮膚科に通院中で,同時期から左眼に羞明があった。所見:左眼に虹彩炎,乳頭の発赤浮腫,網膜血管周囲の白濁がみられた。蛍光眼底造影で網膜白濁部は過蛍光であった。剣創状強皮症に併発したぶどう膜炎と診断した。プレドニゾロン内服とベタメタゾン点眼で徐々に軽快した。結論:剣創状強皮症では眼合併症が併発しやすい。本症例はその例で,副腎皮質ステロイド薬の点眼と内服で軽快した。
Abstract. Purpose:To report a case of infantile localized scleroderma with uveitis. Case:A 4-year-old girl presented with impaired visual acuity in the left eye detected by health-check program. She had been treated by a dermatologist for localized linear scleroderma in the face since 1 year before. Photophobia had been present in the left eye. Findings:The left eye showed signs of iridocyclitis,hyperemia and swelling of the disc,and paravascular opacity in the retina. Fluorescein angiography showed hyperfluorescence in the opaque areas. She was diagnosed with uveitis secondary to localized linear scleroderma. The left eye improved gradually following systemic and topical corticosteroid. Conclusion:The present case illustrates that uveitis may develop secondary to localized linear scleroderma and it may improve following topical or systemic corticosteroid.
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