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Restrictive Dermopathy-A Case Report
Yen-Chi Chou , Wen-Rou Wong , Tseng-tong Kuo , Wei-Hsin Juan, Hong-Shang Hong
Dermatol Sinica 22 : 227-230, 2004
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. |