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[Porphyria cutanea tarda (PCT)].

G Goerz, H Merk

    Zeitschrift Fur Hautkrankheiten
    |January 1, 1985
    PubMed
    Summary

    Porphyria cutanea tarda (PCT) has hereditary and acquired forms, stemming from uroporphyrinogen decarboxylase deficiency. Environmental factors like alcohol and iron can trigger PCT in susceptible individuals.

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    Area of Science:

    • Biochemistry
    • Genetics
    • Dermatology

    Background:

    • Porphyria cutanea tarda (PCT) is classified into two hereditary and one acquired form.
    • Hereditary PCT results from an inherited deficiency in uroporphyrinogen decarboxylase activity.
    • Acquired PCT involves liver-specific enzyme reduction induced by external factors.

    Purpose of the Study:

    • To differentiate between hereditary and acquired forms of Porphyria cutanea tarda.
    • To elucidate the biochemical basis of PCT decompensation.
    • To distinguish PCT from pseudo-PCT.

    Main Methods:

    • Enzyme activity assays for uroporphyrinogen decarboxylase.
    • Analysis of porphyrin profiles.
    • Histopathological examination.

    Main Results:

    • Homozygous hereditary PCT shows severe enzyme deficiency (nearly 5%); hemizygous form has ~50% activity.
    • Heme or porphyrin biosynthesis overload, triggered by ethanol, drugs, or iron, can cause PCT decompensation.
    • Pseudo-PCT, observed in renal failure or with certain drugs, is distinguishable by porphyrin analysis and histology.

    Conclusions:

    • PCT pathogenesis involves uroporphyrinogen decarboxylase deficiency, influenced by genetic predisposition and environmental triggers.
    • Understanding the distinct mechanisms of hereditary, acquired, and pseudo-PCT is crucial for accurate diagnosis and management.
    • Porphyrin analysis and histopathology are key diagnostic tools for differentiating PCT subtypes and pseudo-PCT.

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