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Sultan Qaboos Univ Med J 2023 May 31;23(2):256-258. Epub 2023 May 31.

College of Medicine, Umm Al-Qura University, Makkah, Saudi Arabia.

Primary systemic vasculitis can present with a wide spectrum of manifestations ranging from systemic non-specific features such as fever, malaise, arthralgia and myalgia to specific organ damage. We describe two cases of cholesterol embolisation syndrome and Kaposi sarcoma mimicking primary systemic vasculitis, both of which were characterised by features such as , blue toe syndrome, a brown purpuric skin rash and positive perinuclear anti-neutrophil cytoplasmic antibodies associated with Kaposi sarcoma. Establishing the right diagnosis was challenging and thus this report aimed to highlight the possible ways to distinguish them from primary systemic vasculitis. Read More

Saudi J Kidney Dis Transpl 2021 ;32(5):1489-1494

Department of Nephrology, Command Hospital Air Force, Bengaluru, Karnataka, India.

Cholesterol embolization syndrome is an uncommon complication of coronary angioplasty. Its clinical manifestations are nonspecific and may be ascribed to other causes mimicking vasculitis syndrome. In an appropriate clinical setting, the diagnosis can be confirmed by tissue biopsy. Read More

Am J Cardiol 2022 Mar 2;167:15-19. Epub 2022 Jan 2.

Department of Cardiovascular Medicine, Kurashiki Central Hospital, Kurashiki, Japan.

Cholesterol crystal embolism (CCE) is a rare but serious complication of percutaneous coronary intervention (PCI). However, its incidence, risk factors, and prognosis in the contemporary era are not well known. We included 23,184 patients who underwent PCI in our institution between January 2000 and December 2019 in this study. Read More

Cutis 2021 Mar;107(3):E8-E9

Virginia Commonwealth University, Richmond. Dr. Brinster is from the Departments of Dermatology and Pathology; Dr. Levy is from the Department of Surgery, Division of Vascular Surgery; and Dr. Awadalla is from the Department of Dermatology.

Am J Dermatopathol 2021 Sep;43(9):662-666

Departments of Pathology.

Different hydrophilic and hydrophobic polymers are used as lubricious coatings to reduce vascular traumas in minimally invasive percutaneous procedures. Although they are usually very safe, there is still a risk of serious complications in patients undergoing such procedures, mostly derived from the devices' coating detachment and systemic embolization. The lungs are the most common organ involved, followed by the central nervous system. Read More

J Clin Rheumatol 2021 Mar;27(2):73-79

From the Hospital Universitário Professor Edgard Santos.

In daily practice, the frequent appearance of limb and/or skin necrosis, which we term "acute peripheral and/or cutaneous ischemic syndrome" (APCIS), can be a manifestation of numerous underlying diseases, or it can sometimes be a clinical phenomenon whose etiology is undefined even after a wide investigation. The mechanisms for the development of APCIS include vessel wall abnormalities (atherosclerosis, vasculitis, and calciphylaxis), embolic processes (infectious endocarditis, atrial myxoma, and cholesterol emboli), local thrombotic injuries (genetic or acquired thrombophilias and heparin- and warfarin-induced ischemia), dysproteinemias (cryoglobulinemia and cryofibrinogenemia), or venous limb gangrene. Here, we report 5 illustrative cases of APCIS with different pathogenetic mechanisms, thereby highlighting some clinical conditions that cause APCIS that may be of special interest for rheumatologists, such as antiphospholipid syndrome, primary and secondary vasculitis, and cryoproteinemias. Read More

Rheumatology (Oxford) 2021 Jan;60(1):34-47

Division of Rheumatology, University of California, Los Angeles, Los Angeles, CA, USA.

While prompt diagnosis of vasculitis is important, recognition of vasculitis mimics is equally essential. As in the case of vasculitis, an approach to mimics based on the anatomic size of vessels can be useful. Infections can mimic vasculitis of any vessel size, including the formation of aneurysms and induction of ANCAs. Read More

World Neurosurg 2020 Oct 15;142:274-282. Epub 2020 Jul 15.

Neuroendovascular Therapy Center, Aichi Medical University, Aichi, Japan; Department of Neurosurgery, Aichi Medical University, Aichi, Japan.

Background: The cholesterol embolization syndrome (CES) results from the distal embolization of cholesterol crystals from atheromatous plaques in large vessels such as the aorta and results in multiorgan damage.

Case Description: We present the case of a patient with definite CES with skin manifestations (e.g. Read More

Rev Med Interne 2020 Apr 20;41(4):275-278. Epub 2020 Feb 20.

Service de dermatologie, hôpital René Dubos-Pontoise, 6, avenue de l'île de France, 95300 Pontoise, France.

Introduction: Clinical presentation of cholesterol crystal embolism (CCE) can be dermatologic when cholesterol crystals become lodged in small cutaneous arteries resulting in ischemia. We report a case of CCE with erythroderma misleading to a diagnostic of drug reaction with eosinophilia and systemic symptoms (DRESS).

Case Report: A 66 year-old woman presented with erythroderma few months after initiation of allopurinol. Read More

Rev Port Cir Cardiotorac Vasc 2018 ;25(1-2):77-82

Medicina Geral e Familiar, Unidade de Saúde Familiar São Martinho, Penafiel, Portugal.

Atheroembolism is a rare multisystemic disorder that is characterized by release of cholesterol crystals and particles from atheromatous plaques, which can occlude distal vessels and induce an inflammatory response. Most affected individuals are males, older than 60 years of age, with advanced atherosclerotic disease. The abdominal aorta is the most common origin of cholesterol emboli, being the peripheral arteries a rarer source. Read More

Eur J Dermatol 2017 Apr;27(2):182-183

Department of Dermatology Nagoya University Graduate School of Medicine 65 Tsurumai-cho, Showa-ku Nagoya 466-8550, Japan.

Am J Dermatopathol 2017 Sep;39(9):637-662

*Associate Staff of the Department of Dermatology, Department of Dermatology, Hospital Universitario de la Princesa, Universidad Autónoma, Madrid, Spain; †Associate Staff of the Department of Dermatology, Department of Dermatology, Fundación Jiménez Diaz, Universidad Autónoma, Madrid, Spain; ‡Director of Dermatopathology, Department of Dermatology, Hospital Clínico Universitario, Salamanca, Spain; §Director of Dermatopathology, Dermatopathology Research Unit, Medical University of Graz, Graz, Austria; ¶Director of Dermatopathology, Dermatopathologie Laboratory, Friedrichschafen, Germany; and ‖Chairman of the Department of Dermatology, Department of Dermatology, Hospital Clínico Universitario, Salamanca, Spain.

We review the most characteristic clinical and histopathologic findings of the cutaneous manifestations of the occlusive nonvasculitic vasculopathic disorders. Clinically, most of these conditions are characterized by retiform purpura. Histopathologic findings consist of occlusion of the vessel lumina with no vasculitis. Read More

Clin Dermatol 2016 22;34(2):214-41. Epub 2015 Nov 22.

Department of Dermatology, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA. Electronic address:

There exist a wide variety of multisystem diseases that can affect both the eyes and skin. The skin and eyes may be the initial sites affected, leading to a new diagnosis of a systemic illness, or severe skin and eye involvement can drive treatment for patients with multisystem disease. It is important for physicians to be aware of how to recognize and diagnose these conditions, to evaluate patients for extent of disease, and to initiate appropriate therapies to combat these potentially severe diseases. Read More

Indian J Dermatol Venereol Leprol 2015 ;81(4):388

Department of Medicine, St. Marianna University School of Medicine, Kanagawa, Japan.

Circulation 2015 Feb;131(5):514-5

From Harvard Medical School, Boston, MA (O.A.O.); Harvard Combined Dermatology Residency Program, Boston, MA (K.W.M., D.S., V.E.N.); Department of Internal Medicine, Brigham and Women's Hospital, Boston, MA (V.E.N.); and Departments of Dermatology (M.M.S., A.P.S.) and Pathology (M.P.H.), Massachusetts General Hospital, Boston.

Clin Med (Lond) 2014 Jun;14(3):314-5

Department of Dermatology, School of Medicine, Acibadem University, Istanbul, Turkey

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-specific clinical presentation, which can cause delays in treatment and high mortality. The most common physical symptoms are cutaneous. Read More

Clin Neuropathol 2014 ;33(3):172-8

Simultaneous combined superficial peroneal nerve and peroneous brevis muscle biopsy, via the same cutaneous incision, allows examination of several tissue specimens and significantly improves the diagnosis of systemic diseases with peripheral nerve involvement. Vasculitides are certainly the most frequently diagnosed on neuro-muscular biopsies, but this procedure is also well advised to asses a diagnosis of sarcoidosis or amyloidosis. More occasionally, combined nerve and muscle biopsy may reveal an unpredicted diagnosis of cholesterol embolism, intra-vascular lymphoma, or enables complementary diagnosis investigations on mitochondrial cytopathy or storage disease. Read More

Indian J Surg 2013 Jun 7;75(Suppl 1):432-5. Epub 2012 Oct 7.

Department of Nephrology, Apollo Hospital, Jubilee Hills, Hyderabad, India.

Cholesterol emboli syndrome is due to dislodgment of cholesterol crystals from the atherosclerotic plaques lining the walls of major arteries resulting in an occlusion of small arteries. We describe a case of severe cutaneous and renal cholesterol emboli syndrome following coronary angiography showing our observation that this syndrome is often unrecognized or misdiagnosed and that a better evaluation of risks factors in patients undergoing invasive procedures could prevent this severe complication. Read More

Dermatol Online J 2012 Jul 15;18(7):10. Epub 2012 Jul 15.

Department of Dermatology, Christian Medical College, Vellore, Tamilnadu, India.

An 81-year-old woman with chronic kidney disease, systemic hypertension, and a large infra-renal abdominal aortic aneurysm, developed bilateral calf muscle pain, altered sensorium, and deterioration of renal function following endovascular aneurysmal repair. On the third post-operative day she developed symmetrical purpuric macules with erythematous margins on the gluteal region and bluish reticulated patches on the soles and tips of toes. This was followed by melena development on the seventh post-operative day. Read More

Dtsch Med Wochenschr 2012 May 15;137(21):1130-4. Epub 2012 May 15.

Klinik für Innere Medizin, St.-Antonius-Hospital Eschweiler.

Cholesterol embolization is a serious complication of atherosclerosis. Mainly, vascular manipulations during endovascular procedures or vascular surgery can release cholesterol crystals from atheromatous plaques. About 20 % are spontaneous emboli without precipitating event. Read More

Am J Dermatopathol 2011 Oct;33(7):726-8

Department of Clinical Dermatology, Ospedali Riuniti, Foggia, Italy.

Cholesterol crystal embolization (CCE) is one of the many complications of atherosclerosis. CCE is usually an iatrogenic event occurring either after vascular surgery, usually aortic surgery, or invasive angiographic investigations or in the course of anticoagulant or thrombolytic therapy. More rarely, it occurs after trauma or even in the absence of any inciting cause. Read More

Am J Dermatopathol 2011 Dec;33(8):796-802

Department of Pathology, Lahey Clinic, Burlington, MA 01805, USA.

Ischemic skin necrosis can be a cause of severe morbidity and mortality. It can be due to a number of systemic conditions such as (1) thrombotic vasculopathy syndromes, (2) calciphylaxis, (3) septic or cholesterol emboli, and (4) cutaneous vasculitis. We present 3 patients with a clinicopathological syndrome consisting of ischemic skin necrosis associated with histological pattern of subcutaneous thrombotic vasculopathy-extensive microvascular thrombosis confined to small vessels and capillaries of the subcutaneous tissue. Read More

Surg Clin North Am 2010 Dec;90(6):1161-80

Department of Dermatology, Roger Williams Medical Center, 50 Maude Street, Providence, RI 02908, USA

Skin ulceration is a major source of morbidity and is often difficult to manage. Ulcers caused by an inflammatory cause or microvascular occlusion are particularly challenging in terms of diagnosis and treatment. The management of such ulcers requires careful assessment of associated systemic conditions and a thorough analysis of the ulcer's clinical and histologic findings. Read More

Clin Exp Nephrol 2011 Feb 11;15(1):159-63. Epub 2010 Nov 11.

Nephrology and Dialysis Unit, Hospital São Teotónio-Viseu, Av Rei Dom Duarte, 3504 509 Viseu, Portugal.

Atheroembolic renal disease is caused by foreign-body reaction to cholesterol crystals flushed from the atherosclerotic plaques into the small-vessel system of the kidneys. It is an underdiagnosed entity, mostly related to vascular procedures and/or anticoagulation, and prognosis is considered to be poor. Besides the benefit of aggressive medical prevention of further embolic events, use of steroid therapy has been associated with greater survival. Read More

Nefrologia 2010 22;30(3):317-23. Epub 2010 Apr 22.

Servicio de Nefrología, Hospital 12 de Octubre, Madrid.

Cholesterol embolism is a disease caused by distal showering of cholesterol crystal released from disintegration of arterial atheromatous plaques. It may occur spontaneously or more often after invasive vascular procedures or thrombolytic/anticoagulant agents. Forty five cases were diagnosed between 1989 and 2005 in three Spanish hospitals. Read More

Rev Med Suisse 2010 Mar;6(238):432-4, 436-7

Service de néphrologie et consultation d'hypertension, Département de médecine interne, CHUV, 1011 Lausanne.

Disseminated cholesterol crystal embolism is observed in elderly men with severe atherosclerosis. This syndrome may be triggered by arterial catheterizations, major vascular surgery, thrombolytic and/or anticoagulation treatment. Cutaneous signs, subacute renal insufficiency, a marked inflammatory syndrome and eosinophilia are common. Read More

Lijec Vjesn 2009 ;131(9-10):254-9

Klinika za unutarnje bolesti, Klinicka bolnica Dubrava, Zagreb.

Cholesterol crystal embolism with renal impairment is increasingly recognised as an iatrogenic complication of invasive vascular procedures. We present a 58-year-old patient in whom the presence of a classic triad of precipitating event (coronary angiography), subacute presentation of renal failure and cutaneous lesions (livedo reticularis and Blue Toe syndrome) suggested this entity. The confirmatory diagnosis was made by means of renal biopsy which revealed cholesterol crystals lodged in arteries. Read More

Presse Med 2010 Jun 9;39(6):738-9. Epub 2009 Sep 9.

Université Montpellier I, Service de Dermatologie, Hôpital Saint-Eloi, CHU de Montpellier, F-34295 Montpellier, France.

J Dermatol Case Rep 2009 Aug;3(2):27-9

Department of Plastic and Reconstructive Surgery, Canakkale Onsekiz Mart University, Canakkale, Turkey.

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. Read More

J Cutan Pathol 2010 Jun 10;37(6):692-6. Epub 2009 Jul 10.

Department of Dermatology, Taipei Medical University Hospital, Taipei 110, Taiwan.

Cholesterol embolism (CE) is characterized by emboli containing cholesterol crystal in the arterioles, most commonly found in the skin and the kidney. The skin presentations of CE include livedo reticularis, blue toe syndrome, ulceration and gangrene. Cutaneous reactive angiomatosis (CRA) is a recently proposed term to describe a group of reactive vascular proliferation in skin caused by various diseases. Read More