Cholesterol embolism disease: Report of 16 cases

Background. The cholesterol embolism syndrome is a multisystemic disease resulting from cholesterol crystal embolization to many organs including skin, kidney and CNS. Vascular procedures and anticoagulation have been identified as triggering factors. Patients and methods. Sixteen patients were prospectively reviewed diagnosed of cholesterol embolism syndrome from 1991 to 1998. Results. The mean age was 68 years and all had at least two risk factors for atherosclerosis (hypertension, smoking, diabetes mellitus, hyperlipemia) as well as pre-existing symptomatic atherosclerotic disease. At least one precipitating factor was identified in 14 patients (heparin in 7, coumarins in 4 and vascular procedure in 7). In six patients two or more triggering factors coexisted. Clinically, 12 patients had livedo reticularis, 10 purpuric lesions, 12 purple toes and 4 painful ulcerations. As a result of progressive gangrene 4 patients required amputation of a portion of the lower extremity. The skin biopsy was diagnostic of cholesterol embolism syndrome in 10 cases and was highly suggestive in the remaining cases. Eleven patients developed renal failure but only five required subsequent dialysis. A cerebrovascular accident was reported in two patients and gastrointestinal bleeding occurred in another three patients. Four patients died but only two as a direct result of the disease. Conclusions. The diagnosis of cholesterol embolisms should be considered among elderly patients, with underlying atherosclerotic disease, who develop typical cutaneous manifestations, hypertension, and renal failure in association with precipitating factors. Given the serious implications of this syndrome, a heightened awareness and preventive measures in the population at risk are essential.