Cholesterol embolization syndrome: cutaneous histopathological features and the variable onset of symptoms in patients with different risk factors

Pennington, Mark; Yeager, Josef; Skelton, Henry G.; Smith, Kathleen J.

doi:10.1046/j.1365-2133.2002.04611.x

Cited by 50 publications

(36 citation statements)

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“…Hydration, control of hypertension and haemodialysis has been reported to help lessen organ damage. 10 Although not proven, pentoxifylline, statins and high-dose corticosteroids have been suggested to have a potential benefi t in the treatment of CES. 6,10 …”

Section: Discussionmentioning

confidence: 99%

“…The presence of a classical triad of precipitating events (vascular intervention or procedure), renal insuffi ciency and cutaneous fi ndings mimicking vasculitis suggests a diagnosis of CES. 4,10 The overall mortality rate of CES is 60-87%, and the cause of death is usually multifactorial. 2 Early diagnosis and aggressive supportive care are suggested to be associated with reduced mortality.…”

Section: Discussionmentioning

confidence: 99%

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Lesson of the month 1: Sudden onset postural livedo reticularis, cyanotic toes and multiorgan failure

Duman

Şahin

2014

Clinical Medicine

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Lesson of the month 1: Sudden onset postural livedo reticularis, cyanotic toes and multiorgan failure Cholesterol embolisation syndrome (CES) is a rare but serious disease with high mortality caused by the formation of an embolus made up of cholesterol crystals from atherosclerotic plaques. Its clinical presentation is usually initially insidious and it often remains unrecognised because of its non-specifi c clinical presentation, which can cause delays in treatment and high mortality. The most common physical symptoms are cutaneous. We present a lethal case of CES to increase the awareness about this serious condition. KEYWORDS: Atherosclerotic plaque, embolism, livedo reticularis, cyanosis, multiorgan failure CaseA 65-year-old male presented with a livedoid eruption on the trunk and lower extremities, and cyanosis that had been present for 10 days. He had had a myocardial infarction (MI) 7 weeks earlier. Following the MI, coronary angiography and coronary artery bypass surgery were performed. The patient was being treated with anticoagulant therapy. Dermatological examination revealed cyanosis at the bilateral toes and plantar sides, and livedo reticularis on the bilateral lower extremities and trunk, which became evident after standing for a while (Fig 1-2). Despite cyanotic toes, the patient's peripheral pulses were intact. Laboratory examination revealed acute elevation of serum creatinine at 1.73 mg/dl (0.7-1.2 mg/dl). Histopathological examination of skin biopsy was consistent with a vasculopathic reaction. Cholesterol particles were detected in vitreus during ophthalmological examination. With these clinical fi ndings a diagnosis of cholesterol embolisation syndrome (CES) was made and the patient was hospitalised. Anticoagulant therapy was discontinued, statins were administered and supportive measures were provided. Within 4 days of hospitalisation, the patient was intubated due to the sudden onset of severe dyspnea and haemoptysis. During the patient's time in hospital cardiopulmonary functions ABSTRACT Authors: A dermatology specialist,

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Lesson of the month 1: Sudden onset postural livedo reticularis, cyanotic toes and multiorgan failure

Duman

Şahin

2014

Clinical Medicine

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“…This occurs because: 1) symptoms are often atypical, especially in patients with prominent visceral disease 2) symptoms are frequently not reported by the patients 3) biopsies are often not performed or diagnostic features are not seen, perhaps because sectioning through the block is not routinely ordered when initial sections are diagnostic. 1 Treatment options for CES are limited and nowadays there is no universally accepted pharmacologic treatment. 10 Some authors indicate the need of cessation of anticoagulant therapy with suspicion of the diagnosis.…”

Section: Patient 2 With Cutaneous Cholesterol Embolization Necrotic mentioning

confidence: 99%

“…Panum first described atheromatous embolism in 1862. 1 However, little was known about this syndrome until Flory described the basic pathophysiology of CES in an autopsy report in 1946. The mortality of CES was reported to be 81%.…”

Section: Introductionmentioning

confidence: 99%

Cutaneous Cholesterol Embolization

Avcı

Aköz²,

Gül³

2009

J Dermatol Case Rep

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Background: Cutaneous cholesterol embolization syndrome occurs as a result of cholesterol embolization from atherosclerotic plaques lining the walls of arteries and arterioles. It can occur sporadically but is more commonly associated with iatrogenic manipulation via invasive vascular procedures or therapies (anticoagulation or thrombolytics) The three most common organ systems afflicted by cholesterol emboli include the kidneys, gastrointestinal system, and skin.Main Observations: We present two patients with cutaneous cholesterol embolization. A 74-year-old with no prior disease or trauma was referred to the hospital with the acute chest pain. After 24-48 he developed livedo reticularis, followed by necrotic areas like bilaterally on hips and in the lumbar region. The second patient, 69-year-old man with a history of hyperglycemia, hyperlipidaemia, and hypertension was referred with weight loss, bilateral livedo reticularis, severe pain of lower extremities and impaired renal function. The toes first became cyanotic and than skin necrosis developed. Skin biopsy revealed presence of cholesterol clefts in the lumina of small arteries and arterioles. In both patients the necrotic skin and subcutaneous tissue had been debrided. Daily dressing was applied and tissue defects underwent secondary healing.Conclusion: The diagnosis of cholesterol embolization relies on clinical and histologic examination. Both, diagnosis and treatment needs a multidisciplinary approach, especially in cases of multi-organ involvement.

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“…CES exhibits several cutaneous manifestations, such as livedo reticularis, cyanosis, gangrene and cutaneous ulcers, prior to systemic manifestations including renal failure, abdominal pain and acute pulmonary edema. 2 We present a case of CES with anti-cardiolipin antibody who exhibited livedo reticularis, a cyanotic toe and acute pulmonary edema. …”

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confidence: 99%

Systemic cholesterol embolization syndrome in a patient positive for anti-cardiolipin antibody

Nakamura

Tokura

2010

Dermato-Endocrinology

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Cholesterol embolization syndrome (CES) is a systemic disorder due to a cholesterol crystal embolization from atherosclerotic plaques on the walls of arteries.1 Although CES can take place spontaneously, it often occurs after an invasive vascular procedure. CES exhibits several cutaneous manifestations, such as livedo reticularis, cyanosis, gangrene and cutaneous ulcers, prior to systemic manifestations including renal failure, abdominal pain and acute pulmonary edema. 2 We present a case of CES with anti-cardiolipin antibody who exhibited livedo reticularis, a cyanotic toe and acute pulmonary edema. Case ReportA 76-year-old man presented with an acute pain in his left fifth toe. One month before his visit, he had undergone a heart catheterization for evaluation and treatment of his acute myocardial infarction. Physical examination revealed livedo reticularis on the left foot (Fig. 1A) and cyanotic left fifth toe with purpura (Fig. 1B). The biopsy specimen from the left fifth toe revealed that a small artery in a deep dermis contained biconvex, needle-shaped clefts, hyperplastic intimal proliferation and slight inflammatory infiltrates and erythrocytes within a vessel (Fig. 1C). Laboratory examination demonstrated elevated C-reactive protein (4.65 mg dL -1 ; normal, 0-0.2), slightly elevated serum creatinine (1.28 mg dL -1 ; normal, 0.6-1.1), slightly elevated blood urea nitrogen (25 mg dL -1 ; normal, 8-22) and normal leukocyte count (7,800 μL -1 ; normal, 3,100-9,100) and platelet count (260,000 μL -1 ; normal, 157,000-340,000). Further examination disclosed that anti-cardiolipin antibody was present, while anti-β2-glycoprotein-1 antibody was not detected. Anti-nuclear antibody was within normal limit. One week after the first visit to our hospital, the patient noticed an abrupt chest pain and radiographic examinations revealed an Cholesterol embolization syndrome (CES) is caused by a cholesterol crystal embolization from atherosclerotic plaques on the walls of arteries. it often occurs after an invasive vascular procedure. CES exhibits several cutaneous manifestations before systemic symptoms take place. here, we report a case of CES, positive for an anti-cardiolipin antibody, with a history of cerebral infarction. the existence of anti-phospholipid antibody may be a risk factor for CES after an invasive vascular procedure.Systemic cholesterol embolization syndrome in a patient positive for anti-cardiolipin antibody motonobu nakamura* and Yoshiki tokura Department of Dermatology; university of occupational and Environmental health; Fukuoka, Japan Key words: systemic cholesterol embolization syndrome, anti-phospholipid antibody syndrome, anti-cardiolipin antibody acute pulmonary edema. After the systemic administration of corticosteroid, both the pulmonary edema and livedo reticularis on the foot subsided gradually. Based on the clinical and laboratory findings, we diagnosed him as having CES associated with anti-cardiolipin antibody. DiscussionAnti-phospholipid antibodies, including anti-cardiolipin antibodie...

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Cholesterol embolization syndrome: cutaneous histopathological features and the variable onset of symptoms in patients with different risk factors

Cited by 50 publications

References 28 publications

Lesson of the month 1: Sudden onset postural livedo reticularis, cyanotic toes and multiorgan failure

Lesson of the month 1: Sudden onset postural livedo reticularis, cyanotic toes and multiorgan failure

Cutaneous Cholesterol Embolization

Systemic cholesterol embolization syndrome in a patient positive for anti-cardiolipin antibody

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