Presentation of Ehlers-Danlos syndrome: iliac artery pseudoaneurysm rupture

We present a case of acute flank and groin pain resulting from a ruptured left iliac artery with pseudoaneurysm in a patient with previously undiagnosed Ehlers-Danlos syndrome (EDS). EDS is an uncommon, heterogeneous disease of abnormal collagen production with potentially fatal sequelae. The nine major types of EDS have varying clinical manifestations and degrees of severity. Type IV EDS is of special concern in that it is frequently associated with life-threatening vascular complications such as arterial aneurysm formation with rupture and dissection. EDS should be suspected in young people with vascular complications, recurrent joint dislocations, or fragile skin. In patients with known EDS, abdominal, flank, or pelvic pain should be investigated promptly to rule out life-threatening complications involving the vascular system, gastrointestinal tract, or gravid uterus.