Langeron P, Coisne T
Sem Hop. 1979;55(5-6):292-5.
A man of 40 years of age with the Ehlers-Danlos syndrome was noted to have an arteriopathy characterized by a rectilinear aspect of the iliac trunks with stenosis of the external iliac. Macroscopic examination showed thickening of the tunica of the arterial walls, while an increase in the elastic fibers in the intima was observed on histological examination. This type of arteriopathy appears to be extremely rare in the Ehlers-Danlos syndrome but these findings show similarities between this case and certain arterial affections of systematized elastorrhexis. The possible relationships between arteriopathy and elastic dysplasia are discussed as well as the classification of this very particular type of arteriopathy reported.