Idiopathic scaphoid avascular necrosis in a patient with hypohidrotic congenital ectodermal dysplasia

Idiopathic scaphoid avascular necrosis in a patient with hypohidrotic congenital ectodermal... Hypohidrotic ectodermal dysplasia (HED), also called Christ–Siemens–Touraine (CST) syndrome, is a rare genetic syndrome that affects structures of ectodermal origin, such as the nails, teeth, hair, sweat glands, and skin. Deficiency or absence of these anatomical structures can result in hypotrichosis, hypodontia, hypohidrosis, or anhidrosis. Most cases show X-linked inheritance associated with mutations in the ectodysplasin ( EDA ) gene, although autosomal dominant or recessive inheritance patterns have also been observed. Avascular necrosis of the scaphoid bone is common after a fracture and can also be associated with systemic disease or chronic steroid administration, while idiopathic avascular necrosis of the scaphoid is very rare. This report presents a patient with HED who developed idiopathic avascular necrosis of the scaphoid and discusses the potential association between the two. Level of Evidence: V, diagnostic study. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png European Journal of Plastic Surgery Springer Journals

Idiopathic scaphoid avascular necrosis in a patient with hypohidrotic congenital ectodermal dysplasia

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Publisher
Springer Berlin Heidelberg
Copyright
Copyright © 2015 by Springer-Verlag Berlin Heidelberg
Subject
Medicine & Public Health; Plastic Surgery
ISSN
0930-343X
eISSN
1435-0130
D.O.I.
10.1007/s00238-014-1032-7
Publisher site
See Article on Publisher Site

Abstract

Hypohidrotic ectodermal dysplasia (HED), also called Christ–Siemens–Touraine (CST) syndrome, is a rare genetic syndrome that affects structures of ectodermal origin, such as the nails, teeth, hair, sweat glands, and skin. Deficiency or absence of these anatomical structures can result in hypotrichosis, hypodontia, hypohidrosis, or anhidrosis. Most cases show X-linked inheritance associated with mutations in the ectodysplasin ( EDA ) gene, although autosomal dominant or recessive inheritance patterns have also been observed. Avascular necrosis of the scaphoid bone is common after a fracture and can also be associated with systemic disease or chronic steroid administration, while idiopathic avascular necrosis of the scaphoid is very rare. This report presents a patient with HED who developed idiopathic avascular necrosis of the scaphoid and discusses the potential association between the two. Level of Evidence: V, diagnostic study.

Journal

European Journal of Plastic SurgerySpringer Journals

Published: Apr 1, 2015

References

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