原発性肝癌を併発した晩発性皮膚ポルフィリン症の1例

概要

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We report a case of porphyria cutanea tarda (PCT), who developed hepatocellular carcinoma seven years after the onset of PCT. A 49-year-old male was admitted to our hospital in 1982 because of itching and jaundice. Diagnosis of PCT was made on the base of long-term alcoholic ingestion, liver dysfunction, histological findings of skin biopsy sample and marked urinary excretion of uroporphyrin and coproporphyrin. Deferoxamine was effective but itching and jaundice again appeared seven years later and he was readmitted. He was markedly jaundiced and died soon after the admission of hepatocellular carcinoma which was confirmed at autopsy. Deposition of fat and iron were demonstrated in the non-carcinomatous tissues. The pathogenic mechanism of the occurrence of hepatocellular carcinoma in PCT is still a problem to be solved.
社団法人日本肝臓学会の論文