The Cutaneous Manifestations of Cholesterol Crystal Embolization

Abstract

In an effort to provide a greater understanding of the cutaneous features of cholesterol crystal embolization (CCE), we reviewed the clinical features of all histologically proved cases reported in the English-language literature, exclusive of cases in which there was only central nervous system or cardiac involvement. We found that the skin is involved in 35% of patients with CCE. Livedo reticularis is the most common skin manifestation (49%), followed by gangrene (35%), cyanosis (28%), ulceration (17%), nodules (10%), and purpura (9%). Cholesterol crystal embolization was most common in older men and was frequently associated with anticoagulant therapy (36%) or vascular procedures (31%). Because of its protean systemic manifestations, CCE was often mistaken for vasculitis (16%) and periarteritis nodosa (12%). Mortality was very high (72%). Histologic confirmation of cutaneous CCE was possible in 92% of cases where a skin biopsy specimen was obtained. We conclude that the diagnosis of cutaneous CCE should be suggested and easily confirmed histologically in the appropriate clinical setting.

A woman with purple toes after intrarticular hyaluronic acid administration

“…plain the very unusual presentation of the blue toe syndrome described. The clinical picture of blue toe syndrome presents with lower extremity pain, livedo reticularis, purpura, cyanosis, "purple toes", and cutaneous necrosis (11). Fever, myalgia, weight loss, gastrointestinal bleeding, vascular bruits, encephalopathy, altered mental state, and pancreatitis could also be seen in the extended disease (12).…”

Cutaneous Cholesterol Embolization

“…When cholesterol emboli are released into the bloodstream, they travel distally until they lodge in small arterioles that characteristically range from 100μm to 200μm in size. 3 With the aorta being the leading site of atherosclerosis, the downstream sites of kidneys, viscera and lower extremities show the highest frequency of involvement. Pedal pulses are generally normal, absent in 19% and unilaterally absent in 2% of patients.…”

“…A prior coronary angiogram was reported in 4% of cases. 3,4 Cholesterol embolization also has been associated with major vessel surgery, angioplasty intra-aortic balloon pump placement, anticoagulation, thrombolytic therapy and cardiopulmonary resuscitation. 5 Thrombolytic therapy and anticoagulants appear to precipitate cholesterol emboli by dissolving protective thrombi and fibrin deposits coating an atheromatous plaque, permitting the release of cholesterol.…”

“…9 Other dermatologic manifestations of cholesterol embolism consists of ulceration of the legs and feet, purpura and nodular lesions, suggestive of polyarteritis nodosa, and penile and scrotal necrosis. 3 The multiple organ involvement of CES often mimics a systemic vasculitis or a connective tissue disorder. The differential diagnosis in patients with CES often includes both autoimmune or pauci-immune vasculitides and septic embolism.…”

Caso nº 6/98 - Instituto Dante Pazzanese de Cardiologia - São Paulo

“…As biópsias no microateroembolismo são feitas, geralmente, em lesões cutâneas e devem incluir porções de tecido muscular esquelético, visando ao diagnóstico diferencial com vasculites necrotizantes de médios e pequenos vasos, como aquelas do grupo da panarterite nodosa (PAN) [3][4][5] . A detecção de ateroêmbolo nos pequenos vasos sela o diagnóstico de microateroembolismo, porém a possibilidade desta demonstração é casual, resultando em variada sensibilidade deste método auxiliar.…”