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Treatments and drugs

By Mayo Clinic staff

Acute porphyrias
Treatment of acute porphyrias focuses on eliminating symptoms. This may require hospitalization in severe cases. Treatment may include:

  • Stopping medications that may have triggered symptoms
  • Medication to control pain
  • Prompt treatment of infections or other illness that may have caused symptoms
  • Intravenous sugar (glucose) to maintain an adequate intake of carbohydrates
  • Intravenous fluids to combat dehydration
  • Hemin or hematin (heme arginate) injections, medication that is a form of heme, which can reduce the burden placed on your body to produce heme, thus lowering porphyrin levels

Cutaneous porphyrias
Treatment of cutaneous porphyrias focuses on reducing the amount of porphyrins in your body to help eliminate your symptoms. This may include:

  • Drawing blood (phlebotomy). Phlebotomy involves drawing a certain amount of blood from one of your veins. This reduces the iron in your body, which decreases porphyrins. You may need to undergo phlebotomy several times before cutaneous porphyria goes into remission.
  • Medication. Drugs used to treat malaria — hydroxychloroquine (Plaquenil) and chloroquine (Aralen) — can absorb excess porphyrins and help your body get rid of them more quickly than usual. These medications are generally used only in people who can't tolerate phlebotomy.
  • Beta carotene. Long-term treatment of cutaneous porphyrias may include daily doses of beta carotene or other carotenoids, such as canthaxanthin. Your body converts beta carotene to vitamin A, which is necessary for healthy eyes and skin. Beta carotene may increase your skin's tolerance to sunlight.
References
  1. Puy H, et al. Porphyrias. The Lancet. 2010;375:924.
  2. Learning about porphyria. National Human Genome Research Institute. http://www.genome.gov/pfv.cfm?pageID=19016728. Accessed March 22, 2011.
  3. Porphyria. National Digestive Diseases Information Clearinghome (NDDIC). http://digestive.niddk.nih.gov/ddiseases/pubs/porphyria/Porphyria.pdf. Accessed March 22, 2011.
  4. Anderson KF. Porphyrias: An overview. http://www.uptodate.com/home/index.html. Accessed March 22, 2011.
  5. Porphyrias. The Merck Manuals: The Merck Manual for Healthcare Professionals. http://www.merckmanuals.com/professional/print/sec12/ch155/ch155a.html. Accessed March 22, 2011.
  6. Desnick RJ, et al. The porphyrias. In: Fauci AS, et al. Harrison's Online. 17th ed. New York, N.Y.: The McGraw-Hill Companies; 2008. http://www.accessmedicine.com/content.aspx?aID=2883658. Accessed March 22, 2011.
  7. Singal AK, et al. Porphyria cutanea tarda and hepatoerythropoietic porphyria. http://www.uptodate.com/home/index.html. Accessed March 22, 2011.
  8. Wolanskyj AP (expert opinion). Mayo Clinic, Rochester, Minn. April 5, 2011.
DS00955 May 7, 2011

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