Which statement correctly describes the management of porphyria cutanea tarda?

Which statement correctly describes the management of porphyria cutanea tarda?

• A. Phlebotomy is a treatment modality.
• B. Iron supplementation is recommended.
• C. Sunlight exposure improves symptoms.
• D. There is no relationship to hepatic disease.

Answer: (A)

Porphyria cutanea tarda is a hepatic porphyria where excess iron in the liver drives overproduction of porphyrins that cause skin photosensitivity. The most effective management is phlebotomy to remove excess iron, which lowers hepatic iron stores and downregulates the steps that produce porphyrins, leading to improvement in skin lesions and photosensitivity. Therapy is usually serial phlebotomies aimed at reducing ferritin to around 20 ng/mL and normalizing transferrin saturation. It’s also important to address liver disease and precipitating factors (such as alcohol use or estrogens) and treat underlying conditions like hepatitis C when present. If phlebotomy isn’t feasible, low-dose hydroxychloroquine can be used to promote porphyrin excretion, but it’s not first-line. Sunshine worsens symptoms, not improves them, and iron supplementation would be harmful, reflecting the link between hepatic iron overload and the condition.

Porphyria cutanea tarda is a hepatic porphyria where excess iron in the liver drives overproduction of porphyrins that cause skin photosensitivity. The most effective management is phlebotomy to remove excess iron, which lowers hepatic iron stores and downregulates the steps that produce porphyrins, leading to improvement in skin lesions and photosensitivity. Therapy is usually serial phlebotomies aimed at reducing ferritin to around 20 ng/mL and normalizing transferrin saturation. It’s also important to address liver disease and precipitating factors (such as alcohol use or estrogens) and treat underlying conditions like hepatitis C when present. If phlebotomy isn’t feasible, low-dose hydroxychloroquine can be used to promote porphyrin excretion, but it’s not first-line. Sunshine worsens symptoms, not improves them, and iron supplementation would be harmful, reflecting the link between hepatic iron overload and the condition.