Cutaneous Cholesterol Embolization

Abstract

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.

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

“…4 Although any organ can be involved in CES, the most commonly affected organ systems include the skin, kidneys and gastrointestinal system. 5 Cutaneous manifestations are observed with a frequency ranging from 35% to 100% in different studies and mostly include livedo reticularis, cyanosis and ulcerations. Occasionally, a few nodules or purpura may ocur.…”

Pigmented purpura and cutaneous vascular occlusion syndromes

“…The lesions present in hours, days, or months. 11 , 33 In total, 20% of cases are spontaneous. 32 Manifestations are noted on the skin in 35-88% of cases as well as in the kidneys and digestive tract, with a variety of cutaneous lesions, such as livedo reticularis in 40%, peripheral gangrene in 35%, cyanosis in 28%, ulceration in 17%, nodules in 10%, and purpura in 9%, along with malaise.…”

“…11 , 32 , 34 Skin manifestations are bilateral and limited to the lower extremities, with normal peripheral pulses. 33 Skin biopsies reveal intravascular clefts that are diagnostic in 92% of cases and correspond to cholesterol crystals that dissolve while fixing the tissue. 11 , 32 The condition carries a poor prognosis with a high mortality rate (81%), secondary to cardiac and renal complications.…”