Cholesterol crystal embolization: an atherosclerotic disease with frequent and varied cutaneous manifestations

Abstract

In this paper the basic pathogenesis of cholesterol crystal embolization (CCE) is described, its clinical characteristics are presented and diagnosis and therapy are discussed. The main focus will be on the cutaneous manifestations; however, considering that CCE is a systemic illness, findings in other organs will also be highlighted, particularly the commonly involved renal and gastrointestinal systems.

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

“…Occasionally, a few nodules or purpura may ocur. [6][7][8] In almost all cases the lower extremities are affected. Livedo reticularis, the most common cutaneous symptom, develops suddenly and bilaterally in the majority of cases.…”

A 60-year-old Man With Bilateral Painful Reticular Patches Of Violaceous Color On The Soles

“…Vascular procedures should generally be avoided. The recommended approach consists of treatment and prevention of cardiac failure and aggressive treatment of metabolic disorders using angiotensin converting enzyme inhibitors, loop diuretics, statins and haemodialysis [3,4]. Pentoxifylline has been reported anecdotally to be beneficial.…”

“…The use of systemic corticosteroids to decrease antiinflamatory response is controversial. Surgery is generally not recommended and only reserved for select cases [3].…”

Cholesterol Embolization Syndrome: How to Recognize and Prevent This Potentially Catastrophic Iatrogenic Disease

“…Patients may be asymptomatic, may present with skin lesions such as blue toe syndrome or may present with renal failure. Iatrogenic events are precipitated by a clear inciting factor (e.g., a vascular procedure), and the time to the onset of clinical disease after the inciting procedure can range from 1 day to several months (6). The clinical manifestations will depend on the organs affected.…”

“…Common signs and symptoms on presentation include skin findings (51%), calf claudication (16%), GI bleeding (15%), weight loss (13%), fever (13%) and retinal cholesterol emboli (11%) (7). Donohue et al (6) reported that, of the cutaneous findings, the most frequent were livedo reticularis (49%), gangrene (35%), cyanosis (28%), ulceration (17%), nodules (10%) and purpura (9%). Almost all involve the lower extremities, and most of the livedo reticularis and cyanosis cases and many of the gangrene cases are bilateral.…”