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An 82-year-old man with chronic kidney disease due to type 2 diabetes, hypertension, bullous pemphigoid, and pneumatosis intestinalis presented to our hospital with a 16-day history of a wound on his left fourth toe. He had no history of toe trauma or heart or vascular diseases or procedures. Physical examination revealed erosions and ulcers on the tip of the left fourth toe, cyanotic fourth and fifth toe tips, and livedo reticularis on the left sole (Fig. 1A). Blood results revealed mild leukocytosis with eosinophilia, renal dysfunction (estimated glomerular filtration rate was 17.15 mL/min/1.73 m2), and cholesterol abnormalities. Blood tests 10 months prior showed an estimated glomerular filtration rate of 31.57, indicating worsening renal function. Skin biopsy of the left sole revealed a small artery in the deep dermis, with luminal narrowing (Fig. 1B) and a cholesterol cleft within it (Fig. 1C).
Figure 1(A) Erosions and ulcers on the tip of the left fourth toe, cyanosis on the fourth and fifth toe tips, and livedo reticularis on the left sole. (B, C) Skin biopsy of the left sole region reveals a small artery with narrowing of the lumen in the deep dermis (B, arrow, hematoxylin & eosin, × 4). A cholesterol cleft is observed in the vessel (C, arrow, hematoxylin & eosin, × 20).
The patient was diagnosed with cholesterol embolization syndrome. Intravenous methylprednisolone sodium succinate and broad-spectrum antibiotics were initiated on the day of admission, and low-density lipoprotein cholesterol apheresis was initiated the following day due to further deterioration. After apheresis, the patient's toe findings improved and renal function returned to baseline. He was discharged 12 days after admission.
Discussions
Cholesterol embolization syndrome starts with the rupture of atherosclerotic plaques in proximal large-caliber arteries, such as the aorta or iliac arteries, followed by embolization of plaque debris and settling of cholesterol crystals in distal small or medium arteries.
Blood tests often indicate inflammation and eosinophilia. Biopsies of the involved organs (eg, the skin and muscles) may be necessary for definitive diagnosis.
Treatment of cholesterol embolization syndrome includes the management of end-organ ischemia. Corticosteroid administration is assumed to control the secondary inflammation associated with cholesterol embolization syndrome.