Restrictive Dermopathy -A Case Report-

拘束性皮膚病變－一病例報告－

10.29784/DS.200409.0004

周彥吉(Yen-Chi Chou)；翁雯柔(Wen-Rou Wong)；郭承統(Tseng-Tong Kuo)；阮維鑫(Wei-Hsin Juan)；洪宏翔(Hong-Shang Hong)

Restrictive dermopathy ； Genodermatosis

中華皮膚科醫學雜誌

22卷3期（2004 / 09 / 01）

227 - 230

英文

我們報告一拘束性皮膚病變的31週出生嬰兒，其為一罕見的，體隱性遺傳皮膚病。患嬰有著薄，緊貼的皮膚，造成關節臠縮及呼吸不全。皮膚的組織學檢查發現包括平坦的真皮－表皮交界，變薄的真皮有著緊密排列的膠原纖維，及發育不全的附屬器構造。

We report an infant delivered at 31 weeks gestation with restrictive dermopathy, which is a rare autosomal recessive genodermatosis. The baby had thin, tightly adherent skin that causes arthrogryposis and respiratory insufficiency. Histological findings of the skin include flat dermal-epidermal junction, thinned dermis with compactly arranged collagen fibers, and hypoplastic appendage structures.