Collagen fibril arabesques in connective tissue disorders.
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.


CONCLUSIONS
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.
Hermanns-Lê
Trinh
T
Department of Dermatopathology, University Hospital of Liège, Liège, Belgium.
Piérard
Gérald E
GE
D000293:Adolescent; D000328:Adult; D000784:Aortic Dissection / Q000473:pathology; D020215:Carotid Artery, Internal, Dissection / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D020405:Dermis / Q000648:ultrastructure*; D004535:Ehlers-Danlos Syndrome / Q000648:ultrastructure*; D004547:Elastic Tissue / Q000648:ultrastructure*; D005260:Female; D005322:Premature Rupture of Fetal Membranes / Q000473:pathology*; D024022:Fibrillar Collagens / Q000648:ultrastructure*; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D011247:Pregnancy; D012008:Recurrence; D012189:Retrospective Studies
D024022:Fibrillar Collagens