The Ehlers-Danlos syndrome: an analysis of the structure of the collagen fibres of the skin.
The structure of the collagen fibres of the skin in the Ehlers-Danlos syndrome (ED-S) was studied in eight patients with ED-S Type I, three patients with ED-S Type II and three patients with the X-linked Type V. The results show that the reducible cross-links are present and undergo the same maturation process to non-reducible cross-links as in normal skin. Transmission electron microscopy revealed a normal ultrastructure of the collagen fibrils. At a higher morphological level of organization scanning electron microscopy demonstrated progressive increase in fibre bundle disorder from the X-linked to mitis, to gravis, in which the fibres making up the large fibre bundles demonstrated a considerable inability to aggregate.
D000293:Adolescent; D000328:Adult; D000596:Amino Acids / Q000032:analysis; D002648:Child; D003094:Collagen; D004535:Ehlers-Danlos Syndrome / Q000378:metabolism / Q000473:pathology*; D005260:Female; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D008855:Microscopy, Electron, Scanning; D008875:Middle Aged; D012867:Skin / Q000032:analysis / Q000648:ultrastructure*
D000596:Amino Acids; D003094:Collagen