A case of erythropoietic protoporphyria with early liver cirrhosis improved by chenodeoxycholic acid administration.

症例は35歳男性.1965年より露光部に紅斑・浮腫が出現し,1987年,赤血球中プロトポルフィジン(PP)の高値より造血性プロトポルフィリン症(EPP)と診断された.1994年,肝障害にて当科入院.入院時の血液生化学検査で,AST 63IU/l, ALT 92IU/l, γGTP 303IU/lの肝障害と赤血球中PP値の増加(7,606μg/l)を認めた.肝組織像では小葉構造は乱れ,一部に再生結節が認められた.偏光顕微鏡で重屈折性を示す,褐色色業沈着を肝細胞,Kupffer細胞に認め,以上よりEPPによる早期肝硬変と診断した.ケノデオキシコール酸900mg/日の投与により,ALTは30IU/lと正常化し,γGTP 63IU/l,赤血球中PP5, 748μg/lと低下した.EPPにおいては時に肝不全を呈することがあり,早期診断と肝機能の厳重な経過観察が必要である.本例ではケノデオキシコール酸療法が赤血球中PPの低下と肝機能の改善に有効であったと考えられた.

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