A case of elderly-onset erythropoietic protoporphyria diagnosed at autopsy

The patient was a 71-year-old man. He was diagnosed with autoimmune hepatitis based on the presence of antinuclear antibodies, high immunoglobulin G levels, and a liver biopsy. Prednisolone therapy was ineffective, and the immunosuppressive drugs, such as azathioprine and cyclosporine, were also ine...

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Veröffentlicht in:Kanzo 2023/05/01, Vol.64(5), pp.235-242
Hauptverfasser: Kurosaka, Kazuki, Tanaka, Kosuke, Takai, Miya, Nagasawa, Shigehiro, Morita, Yusuke, Kuwano, Akifumi, Yada, Masayoshi, Narutomi, Fumiya, Hiraki, Yuka, Oishi, Yoshihiro, Masumoto, Akihide, Motomura, Kenta
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Sprache:eng ; jpn
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Zusammenfassung:The patient was a 71-year-old man. He was diagnosed with autoimmune hepatitis based on the presence of antinuclear antibodies, high immunoglobulin G levels, and a liver biopsy. Prednisolone therapy was ineffective, and the immunosuppressive drugs, such as azathioprine and cyclosporine, were also ineffective. Eventually, on day 93, he succumbed to liver failure. Erythropoietic protoporphyria with protoporphyrin accumulation in the liver tissue was discovered following a pathological autopsy. We report a case of liver failure in which the patient was identified with autoimmune hepatitis before his death, but in which autopsy showed a diagnosis of elderly-onset erythropoietic protoporphyria.
ISSN:0451-4203
1881-3593
DOI:10.2957/kanzo.64.235