Ann Dermatol.
2007 Jun;19(2):84-87.
A Case of Restrictive Dermopathy
- Affiliations
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- 1Department of Dermatology, Busan Paik Hospital, College of Medicine, Inje University, Korea. drskin99@paran.com
Abstract
- 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.
