treatment

Last reviewed 01/2018

• treatment of the contributory hepatotoxic agents (for example, abstention for alcohol misuse, venesection for iron overload, and, somewhat controversially, chloroquine therapy) can lead to a useful improvement in the skin lesions, with long term remission in some patients
  • chloroquine
    • forms a complex with uroporphyrin and promotes release of uroporphyrin from the liver
    • may also inhibit the synthesis of uroporphyrin.
  • use of ultraviolet blockers are valuable in management of this condition
• chelation with desferrioxamine is an alternative option where venesection is contraindicated in a patient with iron overload (1)

Reference:

• (1) Poulin V et al. 80-year-old man with anemia and blistering skin lesions. Mayo Clin Proc. 2004;79(9):1181-4
• (2) Thandani H et al. Diagnosis and management of porphyria. BMJ. 2000 Jun 17;320(7250):1647-51