A Case of Restrictive Dermopathy
Annals of Dermatology
; : 84-87, 2007.
Article
em En
| WPRIM
| ID: wpr-158865
Biblioteca responsável:
WPRO
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.
Palavras-chave
Texto completo:
1
Base de dados:
WPRIM
Assunto principal:
Insuficiência Respiratória
/
Pele
/
Colágeno
/
Contratura
/
Derme
/
Epiderme
/
Gordura Subcutânea
/
Articulações
Limite:
Humans
/
Newborn
Idioma:
En
Ano de publicação:
2007
Tipo de documento:
Article