Cholesterol embolisation syndrome T88.8

Embolisation of skin vessels by cholesterol crystals probably from activated, vulnerable arteriosclerotic plaques, which empty spontaneously, after mechanical irritation by angiography, aortic surgery or after therapy with anticoagulants, streptokinase and plasminogen activator.

Occurring spontaneously or as a result of vascular surgery, oral anticoagulation, thrombolytic therapy, angiography or trauma.

Predisposing factors include high blood pressure, hyperlipidemia, diabetes mellitus, peripheral vascular disease, atherosclerosis, aortic aneurysm. The aorta is the most common site of origin of CES. The three most frequently affected organ systems are the kidney, skin and gastrointestinal tract (Burg MR et al. 2025).

The cause is the rupture of arteriosclerotic plaques in an arterial vessel with the release of a conglomerate of cholesterol crystals into the vessel lumen. Such ruptures of unstable, vulnerable plaques with release of cholesterol-containing contents also play a pathogenetic role in myocardial infarction. Released cholesterol emboli migrate distally following the blood circulation until an occulsion of the small arterioles occurs, which typically have a diameter between 100um and 200um.

The sharp crystal structure of the accumulated cholesterol crystals plays a significant role in the rupture. On the one hand, these cause local inflammation and thus support the inflammatory process in the plaques (unstable plaque). In a broader sense, this mechanism can be compared with the inflammation in uric acid deposits in gout (Ghanem F et al.(2017). This mechanism must be distinguished from thromboembolism, in which a clot forms at the base of an atherosclerotic plaque, breaks off and leads to an embolism.

The main symptom is the acute onset, localized, sharp (permanent) pain of the skin.

In previously unchanged skin, suddenly appearing, differently sized, bizarrely configured, red-livid patches or plaques (picture of livedo racemosa in about 35% of cases).

As the disease progresses, very painful, chronic and poorly healing, bizarrely defined, often sparsely covered, flat ulcers form. The ulcers prove to be very resistant to treatment.

They are permanently very painful (permanent, localized oxygen deficiency).

Deep excision biopsy in the case of cutaneous involvement.

Imaging examinations such as CT, angiography, MRI are helpful in identifying the source of the embolus.

Renal function diagnostics (GFR, cretinin, urine tests)

Ophthalmologic consultation (exclusion of retinal cholesterol embolism)

Symptomatic: pain therapy.

Furthermore aspirin/clopidogrel; possibly high-dose statins gfls. combined with short-term use of glucocorticosteroids.

Notice! Important is the treatment of the underlying internal disease.

The skin infarcts of the cholesterol-embolism syndrome are only a (possibly monitoric) partial manifestation of a systemic arteriosclerotic"embolic disease". Target organs are besides skin, kidneys and brain.

1. Ghanem F et al.(2017) Cholesterol crystal embolization following plaque rupture: a systemic disease with unusual features.J Biomed Res 31:82-94.

2. Burg MR et al.(2025) Occlusive cutaneous vasculopathies: rare differential diagnoses. J Dtsch Dermatol Ges 23:487-506.

3. Gibbs MB et al (2005) Livedo reticularis: an update. J Am Acad Dermatol 52: 1009-1019
4. Lukacs A et al (1992) Cutaneous ulcerated cholesterol embolism under the image of livedo racemosa after cardiac catheterization. Act Dermatol 18: 14-16
5. Panum PL (1862) Experimental contributions to the doctrine of embolism. Virchows Arch 25: 308-310
6. Pennington M, et al. (2002) Cholesterol embolization syndrome: cutaneous histopathological features and the variable onset of symptoms in patients with different risk factors. Br J Dermatol 146: 511-517
7. Resnik KS (2003) Intravscular cholesterol clefts as an incidental finding. Am J Dermatopathol 25: 497-499
8. Scolari F et al. (2000) Cholesterol crystal embolism: A recognizable cause of renal disease. Am J Kidney Dis 36: 1089-1109