Background and purpose:The etiopathogenesis of spontaneous cervical artery dissection (sCAD) is largely unknown. Electron microscopic (EM) examination of skin biopsies of sCAD patients revealed very subtle pathologic changes of dermal connective tissue in about half of these patients leading to the hypothesis of an underlying connective tissue disorder. However, connective tissue abnormalities did not allow clear discrimination between patients and controls in our hands. Therefore we sought to establish an objective basis for the assessment of connective tissue abnormalities in sCAD patients using standardized morphometric assessment of collagen fibrils.
Methods:We performed a blinded examination of collagen in skin biopsies and sought parameters which are able to discriminate between sCAD patients and controls. Various morphometric parameters were compared between sCAD patients (n=20) and control subjects (n=18).
Results: Previously described “Flower like” collagen fibrils in skin biopsies were extremely rare in patients and controls and did not discriminate between both groups. However, they were abundant in the skin biopsy of a patient with Ehlers-Danlos-Syndrome type III (EDSIII) used as reference. Morphometric parameters like collagen fibril diameter, fibril density and relative fibril area did not discriminate between patients and controls on an individual basis, but the mean diameter of collagen fibrils in the skin was lower in sCAD patients compared to controls while the fibril density was higher resulting in nearly equal fibril areas per unit of area (relative fibril area) comparing both groups as well as individuals.
Conclusions:Blinded pathological and morphometric analysis of collagen fibers in skin biopsies was in our hands not able to discriminate reliably between sCAD patients and controls on an individual basis but does show differences in collagen fibril morphometry on a group basis. Further, systematic and blinded pathologic studies of skin biopsies in sCAD patients and controls are needed.
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