Multinodular Focal Fatty Infiltration of the Liver in Acquired Porphyria Cutanea Tarda

Abstract

A 42-year-old man presented with a bullous rash of the fingers associated with numerous small milia and malar hypertrichosis. He had a past history of intravenous heroin abuse and of chronic alcoholism. The diagnosis of acquired porphyria cutanea tarda was confirmed by an increased urinary excretion of uroporphyrin. The laboratory tests showed erythrocyte macrocytosis and abnormal serum liver tests (ALAT 131 UI/l; ASAT 73 UI/l; gamma glutamyl transferase, 233 UI/l). Hepatitis C infection with positive HCV-RNA and genotype 1b was found. Abdominal ultrasound showed parenchymal multiple hyperechoic nodules with a marked peripheral hyperechoic rim throughout the liver. These nodules measured about 1 cm and had no mass effect on vascular structures (Fig. 1). The CT-scan (Fig. 2) showed hypodense nodules with central enhancement after iodine contrast injection equivalent to that of the uninvolved liver. On T1-weighted sequences, the MRI scan showed a homogeneous liver, and after intravenous gadolinium injection it showed hyperintense nodular lesions. On T2-weighted sequences, it showed slight hyperintense nodular lesions with a hyperintense millimetric central zone. A percutaneous liver biopsy of a liver nodule was performed under sonographic control and showed mild chronically active hepatitis (METAVIR score A1F1) associated with hemosiderosis and macrovacuolar steatosis. This was consistent with the diagnosis of multinodular focal fatty infiltration of the liver as reported before (1). The patient was treated with phlebotomy and was advised to stop alcohol ingestion.