Cholesterol Embolism or Purple Toe Syndrome

Cholesterol Embolism- is a medical condition in which release of cholesterol in the body becomes responsible for obstruction of blood vessels in different parts of the body. In this article, we will read about the various causes, symptoms, and treatments for Cholesterol Embolism.

Cholesterol Embolism or Purple Toe Syndrome

How Is Cholesterol Embolism or Purple Toe Syndrome Defined?

  • Cholesterol Embolism or Purple Toe Syndrome is also called as Cholesterol Crystal Embolism.
  • Cholesterol aggregation is released from an atherosclerotic plaque and aggregation is known as cholesterol emboli.
  • The cholesterol emboli traverse along the blood steam to different parts of the body.
  • Emboli often obstruct blood vessels. Obstruction of blood vessels causes ischemic (lack of blood supply) illness, which is known as systemic cholesterol embolism.
  • Cholesterol emboli are free cholesterol aggregation dislodged from plaque.
  • The most common symptoms caused by this disorder are skin ischemic symptoms called livedo reticularis along with gangrene formation in the extremities.
  • Obstruction of blood vessels in kidney causes renal failure.
  • Systemic embolus causes obstruction of blood supply to brain, lung and eye resulting in ischemic symptoms and organ dysfunction.

Causes of Cholesterol Embolism or Purple Toe Syndrome

  • Atherosclerotic Embolism1-
    • Cholesterol emboli are dislodged aggregation of cholesterol from atherosclerotic plaque.
    • In about 25% of individuals, Cholesterol Embolism is caused with significant atherosclerosis of large arteries like aorta.
  • Surgical Induced Embolism2-
    • In about 75% of cases, Cholesterol Embolism is caused as a result of a surgical procedure, which involves the blood vessels.
  • Anticoagulant Medications1-
    • Cholesterol Embolism may even develop after administration of anticoagulants or thrombolytic medications, which reduce blood clotting.

Symptoms of Cholesterol Embolism or Purple Toe Syndrome

The symptoms that an individual experiences with Cholesterol Embolism are dependent in majority of the cases on the organs involved.

Non-Specific Symptoms of Cholesterol Embolism or Purple Toe Syndrome

  • Fever
  • Muscle pains
  • Loss of weight
  • Nausea
  • Decreased appetite

Specific Symptoms of Cholesterol Embolism or Purple Toe Syndrome

Lower Extremities Cholesterol Embolism or Purple Toe Syndrome

  • Arterial Pulsation-
    • Obstruction of blood vessels of lower leg causes loss of pulsation in distal part of the leg.
  • Pain-
    • Severe intense pain is experienced in the ischemic (lack of blood supply) leg.
  • Skin Discoloration-
    • Purple Discoloration Of The Skin- Observed in ischemic leg.
    • Gangrene- The distal part of the extremities like toe and feet shows sign of gangrene.

Cholesterol Embolism or Purple Toe Syndrome Affecting Kidneys:

Cholesterol Embolism or Purple Toe Syndrome Affecting Kidneys

  • Renal Failure-
    • Urine output low
    • Fluid retention
      • Edema
      • Pulmonary effusion
      • Enlarged liver
    • Hyperkalemia
      • High potassium
      • Arryhthmia (irregular heart beats)
  • Hypertension

Digestive System Abnormalities Following Cholesterol Embolism or Purple Toe Syndrome-

  • Abdominal Pain-
    • Intense abdominal pain is experienced following embolism of mesenteric arteries.
    • Abdominal tenderness
  • Bowel Obstruction-
    • Nausea and vomiting
    • Distension of abdomen
    • Constipation
    • Paralytic ileus observed on Radiological examination
  • Blood in Stool

Central Nervous System Abnormalities Due To Cholesterol Embolism or Purple Toe Syndrome

  • Cauda Equina Syndrome-
    • Caused by lack of blood supply to lower end of spinal cord
    • Urinary incontinence or being unaware of discharge of urine
    • Bowel Incontinence
  • Polyneuropathy
    • Obstructions of Blood Supply- Blood supply to spinal nerve or segmental nerve is obstructed. Lack of blood supply of group of nerve or spinal nerve causes polyneuropathy.
    • Muscle Weakness- Entire group of muscles or multiple muscles are supplied by segmental or spinal nerve. Lack of blood causes ischemic changes in spinal or segmental nerve resulting in muscle weakness or paralysis.
    • Sensory Symptoms- Lack of blood supply causes necrosis (cellular death) of sensory nerve, which results in numbness of the skin supplied by the nerve.
  • Mononeuropathy
    • Ischemia of Single Nerve- Causes lack of blood supply to nerve
    • Muscle Weakness- Muscle supply by the ischemic nerve become weak or paralyzed.
    • Sensory Symptoms- Symptoms like tingling and numbness is observed along the ischemic nerve.
  • Ischemia of Brain-
    • Symptoms of Stroke-
      • Unconscious
      • Convulsion

Pulmonary Embolism3- Emboli blocks pulmonary artery.

  • Cough
  • Fever
  • Chest pain
  • Short of breath (dyspnea)

Eyes- Effects of cholesterol embolism on eye.

  • Blockade of retinal artery causes blindness unilateral or bilateral.4
  • Diplopia

Diagnosis of Cholesterol Embolism or Purple Toe Syndrome

Standard investigations like routine blood draws do not clearly confirm the diagnosis of Cholesterol Embolism hence its diagnosis is usually difficult.

Blood Examination-

  • C-Reactive Protein (CRP)- Elevated
  • Erythrocyte Sedimentation Rate (ESR)- Elevated.
  • Blood Urea Nitrogen and Creatinine Level- Elevated.

Urine Examination- Abnormal urine examination suggests possible emboli of renal (kidney) blood vessels.

  • Hematuria
  • Proteinuria
  • Low urine volume
  • Pain- Flank dermatome (area).

Arteriogram

  • Study of blood supply to organ involved is a diagnostic test.

Biopsy

  • Biopsy of the organ shows the ischemic changes and also shows presence of intravascular cholesterol crystals.

Treatment for Cholesterol Embolism or Purple Toe Syndrome

The frontline treatment for Cholesterol Embolism is usually symptomatic in the event of a kidney failure due to Cholesterol Embolism or Purple Toe Syndrome; Statins have been shown to be useful and cut down the risk for hemodialysis by about 50%.

Non-Specific Treatment for Cholesterol Embolism or Purple Toe Syndrome5

  • Nutritional support
  • Fluid management
  • Pain management
  • Low dose prednisone or corticosteroids6

Specific Treatment For Cholesterol Embolism or Purple Toe Syndrome

  • Pulmonary Embolism-
    • Mechanical ventilation
    • Pain medication
    • Treat infection with antibiotics
  • Kidney Embolism-
    • Fluid balance,
    • Treat arrhythmia (irregular heart beats) caused by hyperkalemia (high blood potassium level)
    • Dialysis

Surgical Treatment For Cholesterol Embolism or Purple Toe Syndrome

  • Angioplasty

References

1. Cholesterol microembolization syndrome: a complication of anticoagulant therapy.

Varis J1, Kuusniemi K, Järveläinen H.

CMAJ. 2010 Jun 15;182(9):931-3.

2. Cholesterol crystal embolization diagnosed on bladder transurethral resection.

Chatelain D1, Cordonnier C, Brevet M, Petit J, Sevestre H.

Ann Diagn Pathol. 2005 Aug;9(4):231-3.

3. Pulmonary cholesterol crystal embolization.

Sabatine MS1, Oelberg DA, Mark EJ, Kanarek D.

Chest. 1997 Dec;112(6):1687-92.

4. Retinal emboli in cholesterol crystal embolism.

Rousseau A1, de Monchy I1, Barreau E1, Yahiaoui Y2, M'garrech M1, Kaswin G1, Labetoulle M1.

Case Rep Ophthalmol Med. 2013;2013:421352. doi: 10.1155/2013/421352. Epub 2013 Dec 11.

5. Cholesterol crystal embolism: diagnosis and treatment.

Meyrier A.

Kidney Int. 2006 Apr;69(8):1308-12.

6. Low-dose prednisolone ameliorates acute renal failure caused by cholesterol crystal embolism.

Nakayama M1, Nagata M, Hirano T, Sugai K, Katafuchi R, Imayama S, Uesugi N, Tsuchihashi T, Kumagai H.

Clin Nephrol. 2006 Oct;66(4):232-9.

Written, Edited or Reviewed By:

, MD, FFARCSI

Last Modified On: September 17, 2016

Pain Assist Inc.

Pramod Kerkar
  Note: Information provided is not a substitute for physician, hospital or any form of medical care. Examination and Investigation is necessary for correct diagnosis.

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