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Collagen Fibril Arabesques in Connective Tissue Disorders

  • Original Research Article
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American Journal of Clinical Dermatology Aims and scope Submit manuscript

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.

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Acknowledgments

This work was supported by a grant from the Fonds d’Investissement de la Recherche Scientifique of the University Hospital of Liege. No other sources of funding were used to assist in the preparation of this study. The authors have no conflicts of interest that are directly relevant to the content of the study.

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  1. Department of Dermatopathology, University Hospital of Liège, CHU Sart Tilman, B-4000, Liège, Belgium

    Trinh Hermanns-Lê &  Gérald E. Piérard

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  1. Trinh Hermanns-Lê
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  2. Gérald E. Piérard
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Hermanns-Lê, T., Piérard, G.E. Collagen Fibril Arabesques in Connective Tissue Disorders. Am J Clin Dermatol 7, 323–326 (2006). https://doi.org/10.2165/00128071-200607050-00006

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