The patient that led the authors to make some physiopathological reflections on the Ehlers-Danlos syndrome (EDS) was a young 30 year old suffering from a poly-aneurismatic syndrome, identifiable as EDS type IV.
After a surgical treatment on the abdominal aortic aneurysm and an aorto bis-iliac by-pass, a specimen of the aortic wall was the subject of an ultrastruc tural study.
In particular, correlations between the histopathological alterations were sought.
Particular reference was made to the collagen and the clinical vascular mani festations found not only in this patient but also repeatedly in other patients suffering from this type of EDS.
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References
1.
Barabas AP: Vascular complications in the Ehlers-Danlos Syndrome. J. Cardiovasc Surg13:160-167, 1972.
2.
Black CM, Gathercole LJ, Bailay AJ, Beighton P.: The Ehlers-Danlos syndrome: an analysis of the structure of the collagen fibers of the skin. Br J Derm102:85-92, 1980.
3.
Byers PH, Holbrook KA, McGillivray B., MacLeod PM, et al: Clinical and ultrastructural heterogeneity of type IV Ehlers-Danlos syndrome. Hum Genet47, 141-150, 1979.
4.
Catrambone G. , Pesce L., Cannata G., lurilli L.: Aspetti chirurgici della malattia di Ehlers-Danlos . Min Chir37:1567-1572, 1982 .
5.
Epstein EH, Jr: Human skin collagen: release by pepsin digestion and preponderance in fetal life. J Biol Chem249, 3225-3231, 1974 .
6.
Gay S., Miller EJ: Collagen in the Physiology and Pathology of Connective Tissue. New York, Gustav Fisher Verlag, 83, 205:1-25, 1978.
7.
Haynes CD, Smith RB, Dempsey RL, Darden W., Jr: Multiple congenital aneurysms associated with spontaneous vascular rupture. Surgery92, 5:910-912, 1982.
8.
Hunter GC, Malone JM, Moore WS, Misiorowsky RC, et al: Vascular manifestations in patients with Ehlers-Danlos Syndrome . Arch Surg117:495-498, 1982.
9.
Lapiere CM, Nusgens BV, Pierard GE: Interaction between collagen type I and type III in conditioning bundles organization . Connect Tissue Res5, 21-29, 1977.
10.
Minor RR: Collagen metabolism. Am J Pathology98, 1, 227-279, 1980.
11.
Pope FM, Martin GR, Lichtenstein JR, et al: Patients with Ehlers-Danlos syndrome type IV lack type III collagen . Proc Natl Acad Sci, USA72, 1314-1316, 1975.
12.
Pope FM, Martin GR, McKusick VA: Inheritance of Ehlers-Danlos syndrome. J Med Genet14, 200-204, 1977.
13.
Pope FM, Jones PM, Wells RS, Lawrence D.: EDS IV (acrogeria): new autosomal dominant and recessive types. J Roy Soc Med73, 180-185, 1980.
14.
Rudd NL, Minrod C., Holbrook KA, Byers PH: Pregnancy complications in type IV Ehlers-Danlos syndrome. The Lancet1/8:50-53, 1983.
15.
Sack G.: Satus sysvascularis, ein Fall von besonderrer Zerreiblichkeit die Blutgefabe . Dtsch Arch Klin Med178:663-669, 1936.
16.
Sandberg LB , Soskel NT, Leslie JG: Elastin structure, biosynthesis and relation to disease states. N Engl J Med304, 566, 1981 .
17.
Wesley JR, Mahour GH, Woolley MM: Multiple surgical problems in two patienst with Ehlers-Danlos syndrome. Surgery87, 3:319-324, 1980.
18.
Wright CB, Lamberth WC, Ponseti IV, Hanson J.: Successful management of popliteal arterial disruption in Ehlers-Danlos Syndrome . Surgery85, 6:708-712, 1979.
