Features:
(1) severe atherosclerosis with ulcerative plaque along the entire length of the thoracic and suprarenal abdominal aorta
(2) bilateral cutaneous atheroembolism
(3) multiple organ involvement from showers of microthrombi and cholesterol emboli
(4) exclusion of severe atherosclerotic peripheral vascular disease as the sole cause, with palpable peripheral pulses in both feet or an ankle blood pressure in at least one leg > 100 mm Hg (nonischemic range for cutaneous gangrene). Most patients will have severe peripheral vascular disease in the lower extremities.
Organs most often showing involvement include:
(1) kidneys
(2) intestines
If the aortic arch is involved, atheroemboli may involve the upper extremities, eyes, brain, skin in the head and neck, or other sites.
Associated findings:
(1) coronary artery disease with poor left ventricular ejection (< 30%) and poorly controlled angina
(2) risk factors for atherosclerosis - smoking, diabetes and hypertension
(3) often an aortic aneurysm
(4) symptoms often poorly controlled by use of antiplatelet drugs
The prognosis is poor. Some patients can treated with an exclusion-ligation procedure in which a axillo-bifemoral reconstruction is performed. A synthetic graft from left axillary artery runs to the left and then right femoral arteries, with ligation of the external iliac arteries.
