A 45-year-old man with porphyria cutanea tarda has a serum ferritin of 480 ng/mL. Alongside stopping alcohol and estrogen exposure, the established first-line therapy that acts by removing the catalytic iron driving uroporphyrinogen decarboxylase inhibition is:

• A High-dose parenteral desferrioxamine as primary therapy
• B Serial phlebotomy until ferritin approaches the lower normal range ✓
• C Oral isotretinoin daily for six months
• D Plasmapheresis every two weeks

Explanation

Iron overload promotes oxidation of uroporphomethene, an inhibitor of hepatic uroporphyrinogen decarboxylase, so regular phlebotomy to deplete iron stores is first-line and usually induces remission. Low-dose hydroxychloroquine or chloroquine is an alternative when phlebotomy is unsuitable. Desferrioxamine is reserved for patients who cannot tolerate venesection and is not standard primary therapy. Isotretinoin and plasmapheresis have no role in porphyria cutanea tarda.

Reference: Harrisons Principles of Internal Medicine, 21st ed.

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