Are You Confident of the Diagnosis?
Characteristic findings on physical examination
Physical examination typically includes a mottled appearance that is similar to livedo reticularis (Figure 1) and cyanosis of the distal extremities. This bluish discoloration of the toes with or without ulceration may serve as a clinical clue to this diagnosis. While it is possible to diagnose this entity on cutaneous exam alone, histologic confirmation is a simple and rapid means of ensuring the correct diagnosis. Ophthalmic examination may be warranted as a minority of patients may have retinal cholesterol crystals.
Expected results of diagnostic studies
Diagnostic testing for cutaneous cholesterol emboli includes histologic examination. This is a straightforward examination as cholesterol crystals are seen within the lumens of small arteries. While histologic criteria are specific, this may or may not be reliable and/or evident in all histologic sections. Thus, communication between dermatologist and dermatopathologist is essential to optimize the chance of detecting these findings.
Further laboratory testing may include complete blood count, complete metabolic panel, and lipid panel; however, these may or may not reveal any useful findings. Findings such as eosinophilia may be present; however, such findings are nonspecific and would only be useful as one of other diagnostic criteria.
The diagnosis of cutaneous cholesterol embolization can be considered with cutaneous examination and confirmed with histologic analysis. Over 80% of clinical diagnoses can be made by cutaneous examination only. This systemic finding is important to diagnosis as it may have significant implications.
The differential diagnosis includes livedo reticularis and other dermatologic conditions that may include this. Fortunately, a biopsy demonstrates cholesterol crystals and thus makes this diagnosis definitive.
Who is at Risk for Developing this Disease?
The frequency of cutaneous cholesterol emboli is increasing and correlates with interventional vascular procedures, angioplasties, and anticoagulant and/or thrombolytic therapies. It can also occur sporadically without any correlation to these treatments.
What is the Cause of the Disease?
The cause of disease is related to cholesterol crystal deposition in small arteries, leading to occlusion. This is most commonly seen with cutaneous arteries; however, visceral involvement is possible and tends to occur more commonly in patients with coexisting renal disease.
Systemic Implications and Complications
The importance of astute recognition and diagnosis of cutaneous cholesterol emboli is highlighted by its potential for multisystem involvement. Three primary causes of death have been determined with this entity: recurrent cholesterol crystal embolization, cardiac failure, and cachexia. Diagnosis can be delayed due to the nonspecific presentation, and thus prompt dermatologic consultation is warranted.
Treatment options are summarized in Table I.
|Stop anticoagulant therapy|
|Corticosteroids (case by case determination)|
|(all used on case by case basis)|
Optimal Therapeutic Approach for this Disease
-Prompt cutaneous diagnosis confirmed by histologic biopsy.
-Consultation with ophthalmology, cardiology, and gastroenterology
-Cessation of anticoagulant therapy
-Possible use of systemic treatment (corticosteroids, ACE inhibitors, statins) based on individual case
The patient should be monitored for improvement of the condition and referred to cardiology for full cardiac evaluation. Laboratory evaluation
should be followed to ensure that lipids are not elevated or improve if they were elevated.
The patient should be educated on the risk factors of this condition andwhat he/she can do to minimize these with lifestyle choices, such as healthy
eating and exercise.
Unusual Clinical Scenarios to Consider in Patient Management
Facial involvement has been reported following a carotid endarterectomy. A case has been reported mimicking vasculitis with associated myeloperoxidase anti-neutrophilic cytoplasmic antibodies, which resolved with the use of intravenous pulse steroid therapy. Cutaneous reactive angiomatosis may accompany cholesterol emboli.
What is the Evidence?
Lane , JE, Lane , TN, Shams , M, Lane , CE. “Cutaneous cholesterol embolization”. J Am Acad Dermatol . vol. 60. 2009. pp. 711-2. (Solitary case report of a case of cutaneous cholesterol embolization. The patient presented as a healthy male with a nonspecific cutaneous eruption that revealed histologic evidence of cutaneous cholesterol embolization. A case report and brief review of the literature is summarized.)
Jucgla , A, Moreso , F, Muniesa , C, Moreno , A, Vidaller , A. “Cholesterol embolism: still an unrecognized entity with a high mortality rate”. J Am Acad Dermatol . vol. 55. 2006. pp. 786-93. (Comprehensive review of cholesterol embolism with review of the literature and examination of 26 patients seen at the authors' institution. Article provides a detailed discussion of diagnosis.)
Kalter , DC, Rudolph , A, McGavran , M. “Livedo reticularis due to multiple cholesterol emboli”. J Am Acad Dermatol. vol. 13. 1985. pp. 235-42. (This article summarizes common cutaneous findings that are associated with cutaneous cholesteraol emboli.)
Belanfant , X, Meyrier , A, Jacquot , C. “Supportive treatment improves survival in multivisceral cholesterol crystal embolism”. Am J Kidney Dis. vol. 33. 1999. pp. 840-50. (This article is the most comprehensive article at this time on the supportive treatment of multivisceral cholesteral crystal embolism. The authors review the diagnosis in detail and offer a wealth of knowledge on diagnostic and treatment pearls for this rare condition.)
Erdin , M, Tezel , E, Biskin , N. “A case of skin necrosis as a result of cholesterol crystal embolization”. J Plast Reconstr Reconstr Aesthet Surg . vol. 59. 2006. pp. 429-32. (A case report of a patient with cholesterol crystal embolization with subsequent skin necrosis. A brief review of the diagnosis is included.)
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