A Case of Restrictive Dermopathy
Annals of Dermatology
;
: 84-87, 2007.
Article
in English
| WPRIM
| ID: wpr-158865
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Respiratory Insufficiency
/
Skin
/
Collagen
/
Contracture
/
Dermis
/
Epidermis
/
Subcutaneous Fat
/
Joints
Limits:
Humans
/
Infant, Newborn
Language:
English
Journal:
Annals of Dermatology
Year:
2007
Type:
Article
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