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KMID : 0032220070190020084
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Annals of Dermatology
2007 Volume.19 No. 2 p.84 ~ p.87
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A Case of Restrictive Dermopathy
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Hwang Seon-Wook
Cho Kyung-Jong
Oh Doo-Jin
Lee Deborah
Kim Jung-Wook
Park Sung-Wook
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Abstract
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Restrictive dermopathy is a lethal, autosomal recessive disorder characterized by tautness of skin, multiple joint contractures, and respiratory insufficiency resulting in fetal akinesia and death during the neonatal period. Histologic findings show a flat dermoepidermal junction, overall thinned dermis with hypoplastic appendages, a dense fibrotic reticular dermis with collagen parallel to the epidermis, and a thick layer of subcutaneous adipose tissue. It is a rare disorder that has not yet been reported in the Korean literature. Herein, we present a case of restrictive dermopathy in a neonate.
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KEYWORD
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Congenital lethal skin disorder, Restrictive Dermopathy
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