A chromatographic study of uroporphyrins from cases of cutaneous, acute and congenital porphyria

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Abstract
Chromatographic methods were applied to the study of urinary uroporphyrins from 7 cases of cutaneous porphyria. The patients, aged between 54 and 68, had suffered for 1-8 yrs. from photosensitivity and bullous skin lesions. None excreted porphobilinogen or suffered from colics or nervous symptoms. In 6 of these cases, the predominant isomer was uroporphyrin I (60-85%) but in 1 there was found approx. 75% of uroporphyrin III. Urinary uroporphyrins from 2 cases of acute porphyria and 2 cases of congenital porphyria were similarly studied for comparison. The former contained 75-80% uroporphyrin III and 20-25% of uroporphyrin I, while in the congenital porphyria material about 95% of uroporphyrin I was found. A porphyrin is descr., the ester of which had a m.p. 211-216[degree], and which behaved as an octa-carboxylic porphyrin on lutidine paper chromatography, but with Rp very slightly greater than that of uroporphyrin, from which it is separable by column chromatography. On decarboxylation, it yielded coproporphyrin III. A hexacarboxylic porphyrin was detected but not isolated. A pentacarboxylic porphyrin was isolated, with an ester of m.p. 210-214[degree]. Paper chromatography by the dioxan method afforded 2 spots of unequal intensity and on decarboxylation coproporphyrin I was produced together with a smaller quantity of coproporphyrin III. The relation of type of porphyria to isomeric pattern of porphyrin excretion is discussed.