Abstract
We report a case of hepatic coproporphyria. He was a 24-year-old male who visited our hospital complaining of abdominal pain and convulsion. Eight months ago, he had suffered from cerebral contusion and had been administered phenobarbital and sodium valproate. On admission, the urine was wine-colored and liver function tests were almost normal. δ-aminolevulinic acid (26 mg/24 hr), porphobilinogen (5.9 mg/24hr), uroporphyrin (1, 296μg/24hr) and coproporphyrin (7, 290μg/24 hr) (I :III = 1 :15.5) of urine, and fecal coproporphyrin (1, 490μg/g dw) were markedly increased. Uroporphyrin and protopor-phyrin of feces, and uroporphyrin, coproporphyrin and protoporphyrin of erythrocyte were not increased. Laparoscopic findings revealed that the liver surface was almost flat, but had a map-like appearance with reddish-brown areas and slightly depressed dark purple areas. Needle biopsy of the liver showed red fluorescence under ultraviolent light. H. E. staining revealed hydropic degeneration and the presence of brown granules (partially positive for iron staining) in the hepatocytes as well as slight lymphocyte infiltration and fibrosis of the Glisson's sheath. Schmorl's staining revealed needle-shaped crystalline enclosures as long as a hepatocytic nucleus.
