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Symptomatic porphyria secondary to hepatocellular carcinoma
Author(s) -
OCHIAI T.,
MORISHIMA T.,
KONDO M.
Publication year - 1997
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1046/j.1365-2133.1997.d01-1159.x
Subject(s) - porphobilinogen deaminase , porphyria , protoporphyrin , photosensitivity , hepatocellular carcinoma , porphyrin , porphobilinogen synthase , heme , acute intermittent porphyria , dehydratase , medicine , protoporphyrin ix , carcinoma , pathology , urinary system , gastroenterology , chemistry , enzyme , biochemistry , physics , photodynamic therapy , organic chemistry , quantum mechanics
Summary A 58‐year‐old woman gave a 6‐month history of porphyria‐like photosensitivity. Fractioned porphyrin analysis by high performance liquid chromatography revealed elevated concentrations of all urinary porphyrins and faecal protoporphyrin. Hepatocellular carcinoma had developed in an otherwise normal liver. Tumour tissue fluoresced strongly under fluorescence microscopy, exhibiting elevated activity of three haem‐biosynthetic enzymes, δ‐aminolevulinic acid (ALA) synthase, ALA dehydratase and porphobilinogen deaminase. This patient did not satisfy any of the criteria for inherited porphyria. The patient's symptoms were relieved after excision of the liver tumour. This strongly suggests that excessive porphyrin synthesis originated from the tumour tissue. Primary porphyria‐like photosensitivity occurs as a paraneoplastic phenomenon, secondary to hepatocellular carcinoma.