Cutaneous Manifestations of Cholesterol Embolism Treatment & Management

Updated: Jun 27, 2019
  • Author: Laura F McGevna, MD; Chief Editor: William D James, MD  more...
  • Print
Treatment

Medical Care

Cholesterol embolization has a serious prognosis. Unfortunately, treatment options remain limited to conservative medical care and cautious surgical intervention. In 1999, Belenfant et al published a prospective study of 67 patients with cholesterol embolism using therapies targeted at the most common causes of death in cholesterol embolism. [2] This approach reduced the 1-year mortality rate to as low as 23%. Since then, in-hospital mortality rates have been reported to be lower when supportive therapy can be optimized. [30]

In general, the optimal approach is to address and remove precipitating factors. If possible, discontinue planned invasive endovascular procedures. The decision to remove anticoagulation remains controversial, and treatment with warfarin may be harmful.

Risk factors must be modified where possible. Manage blood pressure (goal blood pressure < 140/80 mm Hg) using vasodilators (eg, ACE inhibitors, calcium channel blockers, nitrates). Use statin lipid-lowering medications. Aspirin and clopidogrel may be helpful. [31] In patients with laboratory evidence of inflammation (ie, elevation of C-reactive protein and fibrinogen levels, increased erythrocyte sedimentation rate, a change in serum complement levels) corticosteroids may be used.

Institute supportive care. Use high-dose loop diuretics and/or ultrafiltration in patients with pulmonary edema. Provide enteral or parenteral nutritional support.

It must be emphasized that management principles for cholesterol embolism are often conflicting because therapeutic vascular procedures and/or dialysis may aggravate the condition, and these patients tend to be high risk for surgery. Further, there are reports of improvement with anticoagulation. [32, 33] This is likely because damage is not solely from cholesterol crystals, but the clinical picture of thrombosis and vessel obstruction that occur concomitantly and the cascade of inflammation resulting from endothelial damage.

Many published anecdotal reports describe other therapeutic approaches to cholesterol embolism. None has been studied critically; however, these methods may be of some value if surgical intervention cannot be performed or must be delayed.

Case reports exist of spontaneously healing cutaneous lesions.

Individual case reports show benefit from high-dose corticosteroids. Dahlberg et al, [34] Vacher-Coponat et al, [22] Belenfant et al, [2] and others detailed the potential use of steroid therapy for advanced disease, including those with acute renal failure or in those with pronounced cutaneous manifestations. [35] Steroids may limit the inflammatory effects of ischemia and resultant vascular occlusion. Further study is needed to clearly define the role of corticosteroids in the management of cholesterol embolism. Doses have included prednisone at 60 mg/d and methylprednisolone at 80 mg/d, with therapy lasting from 5 days to months, depending on the patient's response.

Multiple case reports have found that low-density lipoprotein (LDL) apheresis with the concomitant administration of other medications has produced favorable clinical outcomes. Simvastatin or alprostadil with LDL apheresis reportedly improves livedo reticularis. [36, 37] LDL apheresis with corticosteroids and/or an angiotensin receptor blocker has been found to decrease skin and brain manifestations, decrease eosinophilia, and improve kidney function.

The 2003 and 2007 studies by Scolari et al [15, 19] underscored the theory that statin therapy may be beneficial in patients with known atheroembolic renal disease. In these patients, statin therapy was associated with a better prognosis (P< .001), even when initiated well after diagnosis. The favorable outcomes associated with statin therapy may be secondary to both the anti-inflammatory and lipid-lowering properties of the 3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase inhibitors, contributing to plaque stabilization and regression, perhaps initiating recanalization.

Successful pain relief and the improvement of purpura, livedo reticularis, and severe cyanosis on the lower extremities have been reported after treatment with intravenous iloprost in 4 cases. [38]

Success has been reported with oral pentoxifylline. [39]

Isolated case reports exist of successful management of cholesterol embolism with combination corticosteroid and cyclophosphamide therapy. [40] Yucel et al noted the treatment of a patient with corticosteroid and cyclophosphamide combination therapy, leading to improvement. [41] The outcome was appreciable improvement in skin lesions, but ultimately the patient was lost to follow up and succumbed to complications associated with infection.

Filip and Dillon reported successful therapy for cholesterol embolism using circulator boot therapy. [42] A circulator boot is a compression boot designed to restore blood flow to areas of ischemia. In the study, 41 legs were treated. Of these, 81% healed completely, 15% improved, and only 2 required amputation. Levels of evidence were not reported. The circulator boot is thought to function by expelling venous and lymphatic columns from the leg, thereby increasing the arterial-to-venous pressure ratio and providing force substantial enough to reperfuse areas of ischemia.

Next:

Surgical Care

The principal goal of surgical treatment of cholesterol embolism is to promptly identify and eradicate the embolic source and to restore arterial flow. Carefully weigh the risks versus benefits of the surgical intervention and/or preoperative arteriography. In select cases, procedural intervention may be a favorable option.

Amputation or resection of infarcted or symptomatic tissues is often required in severe cases. Blue toe syndrome is usually an indication for limb salvage surgery.

Identification of the embolic source and removal of the atheromatous lesions by endarterectomy, a bypass graft, stent grafting, or excision and replacement of the involved segment of aorta may be important in preventing recurrent showers of emboli. In one study of endovascular stent-graft repair of an abdominal aortic aneurysm, resolution of cholesterol embolism was noted in only 2 of 19 patients at 30-day postoperative follow-up. At 1 year, 8 of 9 patients had complete resolution of their ischemic symptoms. [43]

For small uncomplicated aneurysms, intraluminal grafts inserted on a balloon catheter via the transfemoral route may offer an alternative to open surgery.

The role of lumbar sympathectomy to relieve symptoms from ischemic lower extremities in selected patients with blue toe syndrome remains controversial.

Previous
Next:

Prevention

Carefully weigh the risks versus benefits of surgical therapies, and apply the results individually to patients with cholesterol embolism syndrome.

Prevention of recurrent cholesterol embolism may be achieved by discontinuing all forms of anticoagulants.

Identification of the embolic source and removal of atheromatous lesions by endarterectomy, bypass graft surgery, or excision and replacement of the involved segment of aorta may be important in preventing recurrent showers of emboli.

One study of 7621 patients by Eggebrecht et al indicates that use of catheters smaller than 8 French in angiographic procedures may prevent some cases of cholesterol embolism. [44]

Use of distal filters in endovascular procedures is being studied to explore feasibility for procedural effectiveness and prevention of distal embolization. Although these procedures have shown favorable results with the immediate release of cholesterol emboli, it is unlikely that they will prove useful in delayed events. In one study by Holden and Hill, [45] 46 ischemic nephropathic renal arteries underwent renal artery angioplasty and stenting with distal main renal artery protection. In 95% of patients, renal function was stabilized or improved at follow-up. In the control group without distal protection, 25% of patients experienced either unchanged decline or acute deterioration in renal function after the procedure.

Whitlow et al described 75 patients with severe internal carotid artery stenosis who were treated with stents deployed with a distal system protection system. All 75 patients (100%) had grossly visible particulate material aspirated from the filter, and all were without major or minor stroke or death at 30 days. [46]

Siablis et al described 16 patients who underwent lower limb recanalization for both acute and subacute occlusions with distal filter devices. The recanalization rate was 16 (100%) of 16, without any clinical or angiographic evidence of periprocedural distal embolization. [47]

Cardaioli et al reported successful use of filter-assisted stenting in a 70-year-old man.

New strategies for minimizing cholesterol emboli as a result of cardiopulmonary bypass are emerging. One possible preventive measure is off-pump bypass surgery. Lund et al studied cerebral microembolization in 52 patients during cardiopulmonary bypass (29 off-pump). While a greater reduction of cerebral microemboli was noted during off-pump compared with on-pump surgery, clinical outcomes were not significant.

Previous