A case of porphyria cutanea tarda of the liver exhibiting multifocal macrovesicular steatosis in the background of microvesicular steatosis, probably caused by uneven iron accumulation

摘要

A 61-year-old man with chronic hepatitis B and a history of alcohol overconsumption was admitted to our hospital for the scrutiny of multiple echogenic liver nodules. CT and hepatobiliary phase of gadoxetate-enhanced MR imaging revealed no nodular lesions. Quantitative fat fraction images and R2* map of MR imaging suggested homogeneous steatosis and uneven iron deposition in the liver, namely moderately and severely elevated R2* values at the nodules and surrounding background liver, respectively. Biopsy specimens showed macrovesicular fatty liver and less iron deposition at the echogenic nodules, and microvesicular fatty change and more prominent iron deposition at the surrounding liver tissue. The patient's urinary uroporphyrin level was elevated, and the final diagnosis of porphyria cutanea tarda was made. In patients with history of excessive alcohol intake or viral hepatitis, echogenic nodules on ultrasonography along with radiological evidence of absence of space occupying lesions, and presence of excessive intrahepatic fat and iron, might suggest a possible diagnosis of porphyria cutanea tarda.