Heterogeneity of the Ehlers-Danlos syndrome: description of three clinical types and a hypothesis to explain the basic defect(s).

Abstract

Age 20: Rupture of splenic artery; emergency splenectomy Age 25: Large femoral haematoma; required blood transfusion; E.D.S. diagnosed Age 27: Recurrent severe abdominal pain; all investigations nega tive Age 12: Large haematoma in right iliac fossa Age 17: Large haematoma in left popliteal fossa Age 24: Large haematoma in right popliteal fossa; required blood transfusion; E.D.S. diagnosed Age 30: Died of spontaneous rupture of the aorta