Changing patterns in the vascular form of Ehlers-Danlos syndrome
P. Gertsch, P. W. Loup, A. Lochman and P. Anani
We report and analyze two cases of Ehlers-Danlos syndrome (EDS) type 4. The
first manifestation of the disease was a spontaneous perforation of the
colon in a 47-year-old man; he was successfully reoperated on five years
later for the rupture of an abdominal aortic aneurysm. Abdominal pain
demonstrated the syndrome in a 33-year-old woman in whom multiple abdominal
aneurysms were found. A ligation of the anterior tibial artery for
spontaneous rupture was performed five years later. Light and electron
microscopic studies of the skin disclosed similar alterations in both
cases. The diameter of the collagen fiber bundles was reduced and the
diameter of collagen fibrils was increased. It appears that EDS type 4
might be less characteristic than has been previously described.
Classification of the different types of EDS according to electron
microscopy is not possible.
