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A case of restrictive dermopathy

Korean Journal of Pediatrics 2007;50(3):306-310.
Published online March 15, 2007.
A case of restrictive dermopathy
Seung Ik Lee1, Chang Hee Hong1, Yun Ha Cheong1, Mi Seon Kang2, Jong Beom Sin1
1Departments of Pediatrics, College of Medicine, Inje University, Busan, Korea
2Departments of Pathology, College of Medicine, Inje University, Busan, Korea
제한성 피부병증(Restrictive Dermopathy) 1례
이승익1, 홍창희1, 정윤하1, 강미선2, 신종범1
1인제대학교 의과대학 소아과학교실
2인제대학교 의과대학 병리학교실
Correspondence: 
Jong Beom Sin, Email: pedsin@inje.ac.kr
Abstract
Restrictive dermopathy is a rare autosomal recessive disorder in which rigidity or tautness of the skin from the second trimester causes a fetal akinesia deformation sequence (FADS) and early death. Characteristic features include taut skin with prominent subcutaneous vessels, widely open fontanelles and cranial sutures, distinctive facies, flexion contractures, pulmonary hypoplasia, sparse eyelashes and and eyebrows, thin dysplastic clavicles. The histologic abnormalities of the skin are located in a thin dermis, consisting of compactly arranged collagen fibers, scanty elastic fiber. The dermoepidermal junction is flat, and epidermal appendages are absent, miniaturized or immature. The presence of adipose tissue may be increased. We report on the first Korean case of restrictive dermopathy with typical clinical features and histological findings.
Key Words: Restrictive dermopathy , Joint contractures , Skin dysplasia , Fetal akinesia


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