A Case of Cholesterol Embolism Confirmed by Skin Biopsy and Successfully Treated with Statins and Steroids

doi:10.1097/00000441-200605000-00010

The American Journal of the Medical Sciences

Volume 331, Issue 5, May 2006, Pages 280-283

https://doi.org/10.1097/00000441-200605000-00010 Get rights and content

ABSTRACT

Although cholesterol embolism syndrome was recognized as a clinicopathologic entity more than 50 years ago, it is attracting growing attention recently. It is a multisystemic disorder in which cholesterol crystals released from atherosclerotic plaques obstruct small arterioles, resulting in local ischemia and end-organ damage. There are no established treatments, and with the limited treatment options available, it is important to make the diagnosis as early as possible. We present the case of a 68-year-old man with cholesterol embolism who had a few fluttering atheromas in the aorta, as demonstrated by transesophageal ultrasonography. The diagnosis was confirmed by skin biopsy, and treatment with statins and steroids proved effective, as renal failure progressively improved. This case emphasizes the importance of early diagnosis and shows the possible therapeutic effects of statins and steroids for patients with this syndrome.

Section snippets

Case Report

A 68-year-old Japanese man with a long history of hypertension and smoking was admitted to our hospital because of a second episode of blue toe syndrome and progressive renal failure.

The patient had suffered a cerebellar infarction 4 years previously and had been taking warfarin since then. He had suffered the first episode of blue toe syndrome, which affected the right great toe, 1 year previously. He was treated conservatively with beraprost and dermatologic care, which gradually resolved the

Discussion

Although cholesterol embolism syndrome was first recognized as a clinicopathologic entity more than 50 years ago, it is attracting growing attention recently. It can often cause devastating complications, including renal insufficiency, and a triad of precipitating events, such as angiography or anticoagulation therapy, acute or subacute renal failure, and peripheral cholesterol crystal embolization, characterizes this syndrome. Cholesterol crystal emboli detached from the aortic wall, enter the

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Cited by (13)

Cholesterol crystal embolism-related cerebral infarction: Magnetic resonance imaging and clinical characteristics
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Citation Excerpt :
Cholesterol embolism has poor prognosis because it is associated with advanced atherosclerosis, a major risk factor for fatal cerebrovascular events including stroke [18,24]. Although there have been no large-scale studies on specialized treatments for CCE-CI, smaller-scale investigations have documented the efficacy of corticosteroids [25,26] and low-density lipoprotein (LDL) apheresis [27]. Since the treatment of CCE differs greatly from other types of stroke, including ACI, early diagnosis is crucial.
Cholesterol crystal embolism-related cerebral infarction (CCE-CI) is frequently misdiagnosed due to the lack of specific symptoms. To aid in differential diagnosis, this study comprehensively characterized the magnetic resonance imaging (MRI) and clinical manifestations of CCE-CI and compared these features to those of atherothrombotic cerebral infarction (ACI).
This single-center, retrospective, observational study was conducted at Kitasato University Hospital, Kanagawa, Japan. We identified 37 clinically or histopathologically confirmed CCE-CI cases and 110 ACI cases treated from January 2006 to May 2020. Groups were compared for mean age, sex ratio, clinical presentations, imaging manifestations, precipitating factors, comorbid conditions, medications, and smoking history.
Of 37 eligible patients with CCE-CI, 10 (27.0%) received brain MRI, of which 8 (21.6%) exhibited high-intensity signals indicative of brain lesions on diffusion-weighted imaging (DWI). However, two patients with DWI lesions exhibited no detectable neurological abnormalities. Patients with CCE-CI frequently demonstrated bilateral DWI lesions involving the bilateral anterior and posterior circulation, a pattern absent in ACI (50% vs. 0%, p < 0.001). Compared to patients with ACI, CCE-CI patients also demonstrated significantly lower estimated glomerular filtration rate (p < 0.001) as well as more frequent eosinophilia (p = 0.006), atherosclerotic plaques ≥4-mm thick in the ascending aorta or proximal arch (p = 0.001), and aortic aneurysm (p < 0.001).
Patients with CCE-CI develop multiple DWI lesions across several vascular territories, even in the absence of neurological symptoms. Comorbid aortic aneurysm may increase CCE-CI risk. These findings could help in the differential diagnosis of CCE-CI.
Drug-induced cutaneous vasculitis and anticoagulant-related cutaneous adverse reactions: insights in pathogenesis, clinical presentation, and treatment
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Underlying cardiovascular disease is treated with lipid-lowering agents, aspirin or other antiplatelet agents, and blood pressure control. Although treatments such as systemic corticosteroids, anticoagulation with alternative agents, iloprost, and low-density lipoprotein apheresis have been used with variable success, none are widely accepted.130–133 Heparin-induced skin necrosis represents a cutaneous manifestation of a potentially life-threatening systemic process of heparin-induced thrombocytopenia.
Drug-induced vasculitis and anticoagulant-related skin reactions are commonly encountered in the inpatient and outpatient settings. The spectrum of clinical presentation is broad and ranges from focal, skin-limited disease, to more extensive cutaneous and soft tissue necrosis, to potentially fatal systemic involvement. The prompt recognition of these adverse events can have a significant impact on patient morbidity and mortality. We highlight the key features of the clinical presentation with an emphasis on primary lesion morphology, distribution, and epidemiology of purpuric drug reactions. The proposed pathophysiology, histologic findings, and therapeutic interventions of these potentially life-threatening diseases are discussed.
Cholesterol embolization to bladder in setting of transient ischemic attack and hematochezia: An unusual presentation of cholesterol embolization syndrome
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Citation Excerpt :
These treatments are controversial and have not been subjected to randomized trials.3,13 Discontinuation of warfarin and thrombolytic therapy is paramount.3,13,14 Although cholesterol embolization has been described in cases of renal failure and in the abdominal viscera, it has been rarely described in the bladder.
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Cholesterol embolization syndrome following thrombolysis during acute myocardial infarction
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Cholesterol emboli syndrome - A rare complication of cardiac catheterization
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Case ReportsA Case of Cholesterol Embolism Confirmed by Skin Biopsy and Successfully Treated with Statins and Steroids

ABSTRACT

Section snippets

Case Report

Discussion

Am J Kidney Dis

Am J Kidney Dis

J Am Coll Cardiol

Am J Med Sci

Lancet

Cholesterol emboli syndrome

BMJ

Case Reports
A Case of Cholesterol Embolism Confirmed by Skin Biopsy and Successfully Treated with Statins and Steroids