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Ascorbic acid deficiency in porphyria cutanea tarda

P R Sinclair1, N Gorman, S I Shedlofsky

  • 1Veterans Administration Medical Center, White River Junction, Vermont 05009, USA.

The Journal of Laboratory and Clinical Medicine
|August 1, 1997
PubMed
Summary

Plasma ascorbic acid is commonly low in patients with porphyria cutanea tarda (PCT), a condition causing skin photosensitivity. This deficiency appears unrelated to hepatitis C virus (HCV) and may contribute to PCT development.

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Area of Science:

  • Biochemistry
  • Dermatology
  • Hepatology

Background:

  • Porphyria cutanea tarda (PCT) is the most common porphyria, characterized by skin photosensitivity due to excess uroporphyrin and heptacarboxylporphyrin.
  • Animal models suggest ascorbic acid influences uroporphyrin accumulation, hinting at a potential role in PCT pathogenesis.

Purpose of the Study:

  • To investigate whether plasma ascorbic acid levels are decreased in patients diagnosed with porphyria cutanea tarda.
  • To determine if hepatitis C virus (HCV) infection influences ascorbic acid levels in PCT patients.

Main Methods:

  • Plasma samples were collected after an overnight fast from 21 PCT patients (16 with HCV) and 9 patients with HCV but no PCT.
  • Ascorbic acid levels were measured, with 13 PCT patients assessed during active disease and 8 in remission.

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  • Plasma ascorbic acid concentrations were compared between PCT patients, HCV patients without PCT, and healthy controls.

    • Main Results:

    • A significant proportion of untreated PCT patients (85%) exhibited low plasma ascorbic acid levels, with 62% showing deficiency.
    • Two PCT patients with deficient ascorbic acid normalized levels after phlebotomy treatment.
    • Plasma ascorbic acid levels were normal in patients with HCV but without PCT, indicating the decrease in PCT is not solely due to HCV.

    Conclusions:

    • Plasma ascorbic acid concentrations are frequently low in patients with porphyria cutanea tarda.
    • The observed ascorbic acid deficiency in PCT is independent of hepatitis C virus infection.
    • Ascorbic acid deficiency may play a role in the underlying mechanisms contributing to the development of porphyria cutanea tarda.