Erythrocyte uroporphyrinogen decarboxylase activity in 80 unrelated patients with porphyria cutanea tarda

F. KÓSZÓ, M. MORVAY, A. Dobozy, N. SIMON

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Abstract

To estimate the prevalence of the subgroups of porphyria cutanea tarda (PCT), erythrocyte uroporphyrinogen decarboxylase (UD) activity was measured in 80 unrelated patients with PCT, and in 45 of their relatives by using pentacarboxyl‐porphyrinogen III as substrate. The subgroups were differentiated by analysis of the urinary porphyrins of the patients and 119 of their relatives. Of the patients, 77·5% were found to be suffering from the sporadic form of PCT (type I PCT), and 22·5% from the familial form (type II PCT), Every patient with PCT had previously been affected by alcohol, oestrogen or some other liver‐damaging factor. The relative frequency of familial PCT was higher in females (nine of 15) than in males (nine of 65), which suggests that inheritance of the gene for type II PCT may predispose to oestrogen‐precipitated PCT. The onset of type II PCT occurred at a lower age than that of type I (42·6 vs. 47·0 years). The findings suggest an increased risk of precipitating factors in carriers of an inherited UD deficiency.

Original languageEnglish
Pages (from-to)446-449
Number of pages4
JournalBritish Journal of Dermatology
Volume126
Issue number5
DOIs
Publication statusPublished - Jan 1 1992

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Uroporphyrinogen Decarboxylase
Porphyria Cutanea Tarda
Erythrocytes
Precipitating Factors
Porphyrins
Estrogens
Alcohols

ASJC Scopus subject areas

  • Dermatology

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Erythrocyte uroporphyrinogen decarboxylase activity in 80 unrelated patients with porphyria cutanea tarda. / KÓSZÓ, F.; MORVAY, M.; Dobozy, A.; SIMON, N.

In: British Journal of Dermatology, Vol. 126, No. 5, 01.01.1992, p. 446-449.

Research output: Contribution to journalArticle

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