The constellation of multifocal cerebral infarctions, characteristic cutaneous lesions, and acute kidney injury occurring immediately after aortic manipulation strongly supports the diagnosis of CES, highlighting the significant embolic risk associated with endovascular procedures in patients with “shaggy aorta.” Experimental studies have suggested that CES may represent an autoinflammatory condition in which inflammasome pathways involving NLRP3 and IL-1 are activated by cholesterol crystals [12]. The gene discussed is IL1B; the disease is acute kidney injury.