Pseudoporphyria is an uncommon bullous disease with similar clinical and histopathological findings to porphyria cutanea tarda, in the absence of detectable porphyrin elevation. We present a 34-year-old man with clinical and histological findings compatible with porphyria cutanea tarda, whose his urinary uroporphyrin was negative and we concluded that he was a case of pseudoporphyria. Concomita...

5-Aminolevulinic acid is incubated with a crude enzyme extract from Rhodopseudomonas spheroides, mutant R 26. The formed porphyrins (main product: uroporphyrin III) are isolated. Incorporation o f iron, ring-splitting by coupled oxydation and subsequent iron removal leads to a mixture o f pigments, from which urobiliverdin, a new bile pigment with eight carboxylic acid side chains, is isolated....

We describe a method for simultaneously measuring concentrations of coproporphyrin, zinc protoporphyrin IX, and protoporphyrin IX in whole blood by liquid chromatography, with use of reversed-phase ion-pair system, fluorometric detection, and internal standardization. Each analysis requires 10 microL of whole blood and 15 min total analysis time. Analytical recovery ranged from 84 to 92%, day-t...

Coproporphyrin and uroporphyrin are separated from urineand from each other by absorption onto, and Selective elution from, an anion-exchange resin. The two compounds are #{233}eparated by using solvents differing in both polarity and pH. The porphyrins are then quantitated with an Aminco-Bowman Spectrophotofluorometer equipped with an ellipsoidal mirror and a red-sensitive photomultiplier tube...

In 1915 Hans Fischer identified the kinds and types of porphyrins excreted in congenital porphyria (1, 2, 3), and since that time 8 cases of this rare disease have been studied by qualitative, chemical methods (4 to 11). No quantitative studies of the total excretion of porphyrins in this condition have been made, however, and although reports of the therapeutic effect of liver extract on acute...

The ability of drugs to cause uroporphyria in hepatocytes from 17-day-old chick embryos has been investigated and the response of the cells in culture compared with that of the intact liver of the embryos in ovo. In this chick-embryo system, drugs that cause accumulation of uroporphyrin within 19-24 h can only do so in culture; in contrast, 2-allyl-2-isopropylacetamide and 3,5-diethoxycarbonyl-...

Patients with porphyrias have varying degrees of photosensitivity, associated with elevated levels of porphyrins in plasma, erythrocyte, urine and/or feces. To investigate the role of complement in the pathogenesis of cutaneous lesions, varying amounts of uroporphyrin were added to normal human serum (0.1-10 microgram/ml), and the mixtures were then exposed to 405 nm irradiation. Such treatment...