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Cholesterol emboli - Skin
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Cholesterol emboli - Skin

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Contributors: Noah Craft MD, PhD, Lindy P. Fox MD, Lowell A. Goldsmith MD, MPH, Michael D. Tharp MD
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Synopsis

Cholesterol embolization is a disorder in which cholesterol crystals obstruct the vasculature. Multiple organ systems may be involved, but most commonly, kidneys, skin, central nervous system, and gastrointestinal tract are affected. It is characterized by the classic triad of livedo reticularis, acute renal failure, and eosinophilia.

The term cutaneous cholesterol embolism signifies involvement of the skin due to cholesterol embolism. It is also referred to as cholesterol embolization syndrome, lower extremity atheromatous emboli syndrome, and purple or blue toe syndrome.

Cholesterol emboli can occur as a result of invasive coronary procedures such as major vessel surgery, angiography, angioplasty, or following anticoagulant or thrombolytic therapy. Anticoagulants and thrombolytics act by targeting the breakdown of the protective clot that stabilizes an ulcerated atheromatous lesion, thereby releasing cholesterol crystals into the bloodstream. Heparin, warfarin sodium, and streptokinase have been reported as being causative agents. Formation of cholesterol emboli may also follow cardiopulmonary resuscitation or can occur spontaneously.

Embolization occurs when cholesterol crystals splinter off from the atherosclerotic plaques and are carried in the bloodstream to distant organs. The crystals occlude arterioles by 2 mechanisms: first, the crystals physically block smaller arterioles, and second, they activate complement inducing an inflammatory response, which leads to adventitial fibrosis and obliteration of the vessel lumen. Because emboli are small and numerous, this leads to multisystem disease.

Alternatively, larger crystal plaques may break off and directly block larger arteries leading to tissue infarction and acute organ failure. As cholesterol emboli can spread to any organ, they present a wide ranging clinical picture and, hence, diagnosis is difficult. If a patient develops acute pain, skin abnormalities, impaired renal function, hypertension, ischemia in the extremities, or acute multiorgan impairment following an invasive arterial procedure, then a diagnosis of cholesterol embolism should be considered.

Risk factors for the disease are as follows:
  • Extensive atherosclerosis
  • Systemic hypertension
  • Diabetes mellitus
Precipitating factors for the disease are as follows:
  • Angiography
  • Anticoagulation
  • Thrombolysis
  • Vascular intervention such as angiography, heart catheterization, coronary artery bypass grafting, and percutaneous transluminal coronary angioplasty.
  • Elevated levels of plasma C-reactive protein.
In addition to the cutaneous manifestations of the disease, patients will also exhibit the general physical signs of fever, weight loss, tachycardia, and hypertension. Other signs and symptoms will vary according to which system is involved. Cardiovascular system involvement may produce congestive heart failure or a myocardial infarction. If the central nervous system is involved, then stroke, paraplegia, confusion, or delirium may result. Renal involvement occurs quite often, resulting in acute renal failure.

The prognosis is poor, as renal failure is progressive and patients end up requiring dialysis permanently. Ischemic changes often lead to gangrene and subsequent amputation. Death most often occurs from cardiovascular causes, and the mortality rate stands at 78%, as seen in one study. Cholesterol embolism is seen more commonly in males than in females and in whites more commonly than blacks.

Because cholesterol embolization is commonly asymptomatic, it is hard to determine the frequency with which it occurs. However, in one study, incidence of needle-shaped crystals in the lumen of affected arterioles following cardiac catheterization was found to be 10%.

Codes

ICD10CM:
I75.89 – Atheroembolism of other site

SNOMEDCT:
236489002 – Cholesterol embolus syndrome

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

There is a time lag of about 1 to 4 weeks between the causative interventional procedure and onset of the disease, making diagnosis difficult.

The differential diagnosis includes the following:

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Updated: 02/19/2014
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Cholesterol emboli - Skin
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Cholesterol emboli : Abdominal pain, Diarrhea, Dizziness, Fever, Acral, Cyanosis, Gangrene, Oliguria, Weight loss, Ecchymosis, Amaurosis fugax
Clinical image of Cholesterol emboli
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