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Q1: What is the clinical diagnosis? What could be the cause of the ulceration and gangrene in the feet?
The condition is cholesterol crystal embolisation. The ulceration and gangrene in the feet have resulted from catheter manipulation during cardiac angiography and caused occlusion of the small arteries in the feet.
The triad of thigh and foot pain, livedo reticularis, and intact peripheral pulses is considered to be pathognomonic for cholesterol embolisation.
Q2: How can the diagnosis be confirmed?
An eosinophilia, raised erythrocyte sedimentation rate, and decreased complement level may support the diagnosis, however a final diagnosis is only established with a tissue biopsy of the involved skin, muscle, and kidney demonstrating the cholesterol crystals.
Q3: What is the treatment and prognosis?
There is no cure for the disease and the treatment is mainly supportive. The prognosis of the condition is poor with a mortality rate of 72%–80%.
Cholesterol crystal embolisation, is a frequently underdiagnosed clinical disorder. The incidence of this disorder is 0.08%. Though Flory described it as early as 1945, its diagnosis is still a dilemma for clinicians.
This condition predominantly affects the elderly (mean 66 years), frequently those with a history of hypertension (61%), atherosclerotic cardiovascular disease (44%), renal failure (34%), and aortic aneurysm (25%) at presentation.1
Clinical presentation varies and depends upon the origin of the atheroma. Microembolism of the cholesterol crystals and atherosclerotic debris occurs as a result of manipulation by cardiac catheters. If the proximal aorta has been disturbed then symptoms may occur in the central nervous system, abdominal organs, and extremities. The lower extremities are involved if the site is below the renal arteries.
Skin is involved in 35% of patients with cholesterol crystal embolisation. Livedo reticularis is the most common skin disorder (49%), followed by gangrene (35%), cyanosis (28%), and ulceration (17%).2 Other manifestations include transient ischaemic attacks, amaurosis fugax, acute and chronic renal failure,3 and rarer manifestations such as hypertension, bowel ischaemia and infarction, pancreatitis, and adrenal insufficiency. However painful limbs, cutaneous manifestations, and intact peripheral pulses are clinically pathognomonic for cholesterol crystal embolisation.4
Laboratory tests may aid in the diagnosis of cholesterol crystal embolisation. These include eosinophilia, eosinophiluria, raised sedimentation rate, and decreased complement levels. The final diagnosis is established with biopsy and the above mentioned clinical findings.5
Multiple therapeutic regimens have been generally unsuccessful in altering the course of the disease process. The most significant impact on the disease can be made by its prevention. Thrombolytic and anticoagulant therapies are not indicated and only supportive care for such symptoms as hypertension, ulceration, and gangrene is advocated. The prognosis depends upon the extent of the systemic disease and a high rate of mortality (72%–80%) is recorded due to multifactorial, cardiac, and renal aetiologies.1-6
Cholesterol embolisation is a clinical condition occurring after cardiac catheterisation.
Livedo reticularis is the most common skin manifestation followed by gangrene, cyanosis, and ulceration.
Eosinophilia, eosinophiluria, raised sedimentation rate, and decreased complement levels may support its diagnosis. The final diagnosis is only established with biopsy.
It should always be considered in an elderly patient with cutaneous lesions and acute renal failure after an invasive procedure.
Prognosis is poor with a high mortality and the only treatment is supportive.
Cholesterol crystal embolisation.
The authors would like to thank the staff of the library and the research and development department, King George Hospital.