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Journal of Biomedicine and Biotechnology
Volume 2012 (2012), Article ID 878107, 3 pages
http://dx.doi.org/10.1155/2012/878107
Research Article

Dermal Ultrastructure in Low Beighton Score Members of 17 Families with Hypermobile-Type Ehlers-Danlos Syndrome

1Department of Dermatopathology, University Hospital Sart Tilman, 4000 Liège, Belgium
2Department of Rheumatology, Saint-Luc University Hospital, 1200 Brussels, Belgium

Received 13 March 2012; Accepted 12 July 2012

Academic Editor: Richard Tucker

Copyright © 2012 Trinh Hermanns-Lê et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

The distinction between the Ehlers-Danlos syndrome hypermobile type (EDSH) and the benign joint hypermobility syndrome (BJHS) is unclear. The aim of the present study was to compare skin ultrastructural abnormalities of EDSH and BJHS among different families. Skin of 23 EDSH, 27 BJHS, and 41 asymptomatic subjects from 17 families was examined using transmission electron microscopy. Similar ultrastructural abnormalities were found irrespective of the Beighton score. Flower-like collagen fibrils represented the key change and elastic fibers were altered as well. Beighton score is a clinical parameter rating joint mobility that appeared unrelated to quantitative and qualitative collagen ultrastructural alterations in the skin. Some EDSH family members fit with BJHS diagnosis. BJHS possibly represents a mild variant of EDSH.