Collagen fibril arabesques in connective tissue disorders.

Abstract

BACKGROUND:

In various connective tissue disorders, collagen fibrils and elastic fibers may exhibit some ultrastructural abnormalities.

OBJECTIVE:

This electron microscopy study focused on collagen fibril orientation and unusual shapes in the dermis of Ehlers-Danlos syndrome (EDS) and related entities, including spontaneous cervical artery dissection syndrome and recurrent preterm premature rupture of fetal membranes syndrome.

RESULTS:

In addition to some abnormal fibril shapes typically found in these syndromes, other conformations (hook shaped, S shaped, ring shaped) that have not been illustrated previously in the literature were observed.

CONCLUSION:

The abnormal collagen fibril conformations observed in EDS and other related conditions characterized by tissue fragility likely affect both the internal cohesiveness of the bundles and the mechanobiologic properties of the tissue.

• Authors: Trinh Hermanns-Lê, Gérald E Piérard
• Journal: American journal of clinical dermatology (Am J Clin Dermatol) Vol. 7 Issue 5 Pg. 323-6 ( 2006) ISSN: 1175-0561 [Print] New Zealand
• PMID: 17007543 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Fibrillar Collagens

Topics

• Adolescent
• Adult
• Aortic Dissection (pathology)
• Carotid Artery, Internal, Dissection (pathology)
• Child
• Child, Preschool
• Dermis (ultrastructure)
• Ehlers-Danlos Syndrome (ultrastructure)
• Elastic Tissue (ultrastructure)
• Female
• Fetal Membranes, Premature Rupture (pathology)
• Fibrillar Collagens (ultrastructure)
• Humans
• Male
• Microscopy, Electron
• Middle Aged
• Pregnancy
• Recurrence
• Retrospective Studies