Summary
Skin biopsies from 25 patients with the Ehlers-Danlos Syndrome and 15 site, sex and age matched controls were studied by the picrosirius polarization procedure, a specific method for collagen detection in tissue sections. When used in conjunction with histophotometry, this technique permits a quantitative assay of collagen content in the dermis. The light emission measurements obtained showed a statistically significant reduction in the dermal collagen density of Ehlers-Danlos syndrome patients as compared to their controls. Qualitatively, the dermal collagen fibres of these patients appeared frayed and of reduced diameter. These findings indicate that collagen is quantitatively decreased in skin biopsies of patients with the Ehlers-Danlos syndrome.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Black, C. M., Gathercole, L. J., Bailey, A. J. &Beighton, P. (1980) The Ehlers-Danlos syndrome: an analysis of the structure of the collagen fibres of the skin.Br. J. Derm. 102, 85–96.
Holbrook, K. A. &Byers, P. H. (1981) Ultrastructural characteristics of the skin in a form of the Ehlers-Danlos syndrome type IV.Lab. Invest. 44 342–50.
Jansen, L. H. (1955) The structure of the connective tissue, an explanation of the symptoms of the Ehlers-Danlos syndrome.Dermatologica 110, 108–20.
Iunqueira, L. C. U., Bignolas, G. &Brentani, R. R. (1979) Picrosirius staining plus polarization microscopy, a specific method for collagen detection in tissue sections.Histochem. J. 11, 447–55.
Junqueira, L. C. U. &Montes, G. S. (1983) Biology of collagen-proteoglycan interaction.Archs histol. jap. 46, 589–629.
Junqueira, L. C. U., Montes, G. S., Kaupert, D., Shigihara, K. M., Bolonhani, T. M., &Krisztan, R. M. (1981) Morphological and histochemical studies on the collagen in neurinomas, neurofibromas and fibromas.J. Neuropath. exp. Neurol. 40, 123–33.
Junqueira, L. C. U., Zugaib, M., Montes, G. S., Toledo, O. M. S., Krisztan, R. M. &Shigihara, K. M. (1980) Morphologic and histochemical evidence for the occurence of collagenolysis and for the role of neutrophilic polymorphonuclear leukocytes during cervical dilation.Am. J. Obstet. Gynec. 138, 273–81.
Montes, G. S. &Junqueira, L. C. U. (1982) Biology of collagen.Rev. canad. Biol. exp. 41, 143–56.
Pierard, G. E., Pierard-Franchimont C. &Lapiére, C. M. (1983) Histopathological aid at the diagnosis of the Ehlers-Danlos Syndrome, gravis and mitis type.Int. J. Derm. 22, 300–4.
Sulica, V., Cooper, P. H., Pope, F. M. Hambrick Jr., G. W., Gerson, B. M. &McKusick, V. A. (1979) Cutaneous histologic features in Ehlers-Danlos syndrome.Archs Derm. 115, 40–2.
Vogel, A., Holbrook, K. A., Steinmann, B., Gitzelmann, R. &Byers, P. H. (1979) Abnormal collagen fibril structure in the gravis form (type I) of Ehlers-Danlos Syndrome.Lab. Invest. 40, 201–6.
Wechsler, H. L. &Fisher, E. R. (1964) Ehlers-Danlos syndrome. Pathologic, histochemical and electron microscopic observations.Archs Path. 77, 613–9.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Junqueira, L.C.U., Roscoe, J.T. Reduced collagen content and fibre bundle disorganization in skin biopsies of patients with Ehlers-Danlos syndrome. Histochem J 17, 1197–1202 (1985). https://doi.org/10.1007/BF01002502
Received:
Revised:
Issue Date:
DOI: https://doi.org/10.1007/BF01002502