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Porphyria cutanea tarda.

N R Bleasel1, G A Varigos

  • 1Department of Dermatology, Royal Melbourne Hospital, Victoria, Australia.

The Australasian Journal of Dermatology
|December 6, 2000
PubMed
Summary
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Porphyria cutanea tarda (PCT) is a metabolic disorder affecting heme biosynthesis, leading to skin fragility and lesions. Treatments involve avoiding triggers, phlebotomy, and antimalarial drugs.

Area of Science:

  • Biochemistry
  • Dermatology
  • Genetics

Background:

  • Porphyria cutanea tarda (PCT) is a metabolic disorder impacting heme biosynthesis due to reduced uroporphyrinogen decarboxylase activity.
  • PCT manifests with skin fragility, erosions, blisters, milia, and scarring on sun-exposed areas.
  • Excess skin porphyrins reacting with light generate reactive oxygen species, exacerbating skin damage.

Purpose of the Study:

  • To provide a comprehensive overview of Porphyria cutanea tarda (PCT).
  • To discuss the classification, triggers, and clinical manifestations of PCT.
  • To outline current treatment strategies for PCT.

Main Methods:

  • Review of existing literature on Porphyria cutanea tarda.
  • Analysis of the biochemical pathways involved in heme biosynthesis.

Related Experiment Videos

  • Categorization of PCT based on etiological factors (familial, acquired, toxic).

    • Main Results:

    • PCT is linked to decreased uroporphyrinogen decarboxylase activity.
    • Clinical presentation includes photosensitive skin lesions.
    • Identified triggers include alcohol, estrogen, iron, and viral infections.
    • Association with the hemochromatosis gene is noted.

    Conclusions:

    • PCT is a complex metabolic disorder with significant dermatological manifestations.
    • Management requires identifying and withdrawing aggravating factors.
    • Phlebotomy and antimalarial medications are key therapeutic interventions.