Urinary Porphyrin Analyses in Patients With Porphyria Cutanea Tarda

Abstract

Urinary porphyrin analyses were carried out in five patients with porphyria cutanea tarda and in a control group of 44 individuals. Quantitative analysis revealed that the mean value of coproporphyrin was 67.3I.lg/l (range I3.I-I89.Il.lg/l) in 44 normal individuals and the mean value of uroporphyrin was 9.7I.lg/l (range 5.0 to 13.7 I.lg/l) in 11 of 44 controls, but not detectable in the remainder. Four of the five patients with porphyria cutanea tarda, however, had markedly elevated coproporphyrin and uroporphyrin levels. Urinary porphyrin pattern analysis was carried out using one dimentional thin layer chromatography. The pattern in controls revealed that the mean value of coproporphyrin was 85.5%, while the other fractions consituted less than 6.0%. In the porphyria cutanea tarda group, however, hepta-carboxyl porphyrin and uroporphyrin were predominant in two; all five fractions appeared in roughly equal amounts in one; and in the remaining two, coproporphyrin was decreased to a value of only about 50%.A patient treated by phlebotomy was also evaluated by this method. His urinary porphyrin pattern showed a shift from uroporphyrin to a high concentration of coproporphyrin during and after the treatment.With-this method, it is very simple to obtain data for urinary porphyrin pattern analysis, and many samples can be analyzed simultaneously. The profile can aid in the diagnosis of porphyria cutanea tarda, especially when the patients show low levels of urinary porphyrins quantitatively.

Five Cases of Porphyria Cutanea Tarda with Mild Cutaneous Changes: Evaluation of the Efficacy of Phlebotomy by the Pattern Analysis of Urinary Porphyrins

“…However, in some cases, there are reports of a (13). We too described, in a previous report (case 2), a case of PCT in which CP was predominant and the other four porphyrins appeared impartially (12). Case 2 in that report is the same patient as case 3 in this report: the case in which a urinary porphyrin pattern analysis was also performed, showing a predominance of hepta-carboxyl porphyrin and UP.…”

“…Urinary porphyrin, laboratory and histopathological findings are summarized in Tables 1 to 3. Porphyrin analyses were performed using the method previously described (12). A predominance of uroporphyrin was seen in 4 of 5 cases.…”

“…We considered it of great significance that a case of PCT with mild cutaneous changes was presented. In a previous report, it was shown that analysis of the urinary porphyrin profile using thin layer chromatography is feasible and that it indicates the extent of uroporphyrinogen decarboxylase activity (12). In this paper, the urinary porphyrin profile of five cases with PCT are analyzed and the influence of phlebotomy on the urinary porphyrin profile is examined in one case.…”