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Q1: What does the fig 1 show (see p 206)?
Livedo reticularis. This is due to random spasm of cutaneous arterioles with secondary dilatation of capillaries and venules. Livedo reticularis is a common cutaneous manifestation of several conditions such as antiphospholipid syndrome, polyarteritis nodosa, Sneddon's syndrome, dermatomyositis, cryoglobulinaemia, hepatitis C virus infection, amantadine intake, chronic pancreatitis, primary hyperoxaluria, and cholesterol emboli syndrome.
Q2: What kind of crystals are those observed on skin biopsy sample (fig 2, see p 206)?
The skin biopsy specimen shows cholesterol crystals filling the small deep arterial lumen of small arteries.
Q3: What is the final diagnosis?
Cholesterol emboli syndrome.
Cholesterol emboli syndrome is a condition that may be increasingly iatrogenic in origin. Diagnosis is difficult and requires a high index of suspicion, an appropriate clinical picture, and usually, confirmation by biopsy. Although cholesterol emboli occur spontaneously, most patients have undergone an invasive procedure such as diagnostic angiography, percutaneous transluminal coronary angioplasty or cardiovascular surgery, which have the potential for arterial trauma and consequent cholesterol embolisation. The temporal relation of the procedure to the clinical presentation is highly variable, the interval ranging from one day to four months. Anticoagulants and fibrinolytics are also triggering factors for cholesterol embolisation.1 2
The parts of the body most often affected are the kidneys, skin, muscles, and abdominal viscera. Thus, the presence of acute hypertension, renal insufficiency, livedo reticularis, and/or gangrenous skin changes are characteristic manifestations of the multiple cholesterol emboli syndrome.2-4 Ophthalmoscopy may demonstrate bilateral retinal cholesterol emboli.5Laboratory findings that suggest atheroemboli include eosinophilia, a raised erythrocyte sedimentation rate, leucocytosis, and anaemia. Transoesophageal echography may demonstrate ulcerated atheromatous plaques of the thoracic aorta responsible for cholesterol emboli.6
Although the apparent increasing numbers of cholesterol emboli may be a reflection of the increased use of arterial invasive procedures, they are being performed on an older, more severely ill population, with other risks factors for the development of embolic phenomena—for example, age, smoking history, diabetes mellitus, hypertension, and peripheral vascular disease. Thus, there is now growing emphasis on the concept of the triggering factors with the multiplication of endovascular radiological investigations, the more widespread availability of cardiac surgery, and the use of anticoagulants and fibrinolytics.1
Prognosis is related to the extent of systemic involvement but renal disease is particularly threatening and gangrene and infection can be lethal. Patients usually die of multisystem failure. A multiple therapeutic regimen has been generally unsuccessful in altering the course of the disease process. However, supportive treatment can reduce mortality. Treatment includes management of heart failure with vasodilators, loop diuretics, ultrafiltration or haemodialysis; enteral or parenteral nutritional support to avoid cachexia; haemodialysis for severe metabolic disorders, and corticosteroids when there is laboratory evidence of inflammation.7 Isolated reports have recommended the use of pentoxifylline,8simvastatin,9 and prostacyclin analogues.10 However, the most significant impact on the disease can be made by its prevention.
Cholesterol emboli syndrome.
Common clinical presentation of cholesterol emboli syndrome includes renal failure and livedo reticularis in atherosclerotic patients over 60 years of age with a recent history of vascular catheterisation.
Cholesterol emboli syndrome is often unrecognised or misdiagnosed and a better evaluation of risks factors (smoking history, diabetes mellitus, arterial hypertension, peripheral vascular disease) in patients undergoing invasive vascular procedures could prevent this severe complication.
Supportive treatment can improve prognosis but the most significant impact on the disease can be made by its prevention.