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Medication

Medication Summary

Medical therapy for porphyria cutanea tarda may be used alone or in combination with phlebotomy.

Class Summary

These agents are believed to form complexes with porphyrin molecules within hepatocytes that are then discharged into the circulation and excreted by renal mechanisms. Increased urinary iron excretion has also followed their use. Reported experience in treating children with PCT with antimalarials is limited.

Chloroquine (Aralen)

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Chloroquine has anti-inflammatory activity by suppressing lymphocyte transformation and may have photoprotective effects. Chloroquine binds porphyrins and enhances excretion. It is available as a 250-mg tablet and 500-mg scored tablet and is not available as a syrup. Crush the tablet and mask the bitter taste in jam, applesauce, or other soft food.

Doses for chloroquine phosphate (125-250 mg PO twice weekly) are much lower than those used for antimalarial or photoprotective indications. Larger doses can cause severe hepatotoxicity and death. Even low-dose regimens can occasionally produce hepatic toxicity, and careful monitoring is indicated. Some clinicians begin with a single, small test dose. Hepatic transaminases and urinary porphyrin output may rise transiently after institution of therapy, returning to normal levels as treatment continues. Dosing information listed in the full drug monograph for standard antimalarial or other nonporphyria indications is inappropriate for treatment of porphyria cutanea tarda.

Hydroxychloroquine (Plaquenil)

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Hydroxychloroquine inhibits chemotaxis of eosinophils, inhibits locomotion of neutrophils, and impairs complement-dependent antigen-antibody reactions. Doses for hydroxychloroquine sulfate (100-200 mg [77.5-155 mg base] PO 2-3 times/wk) are much lower than those used for antimalarial or photoprotective indications. Hydroxychloroquine sulfate 200 mg is equivalent to 155 mg hydroxychloroquine base and 250 mg chloroquine phosphate. Larger doses can cause severe hepatotoxicity. Even low-dose regimens can occasionally produce hepatic toxicity, and careful monitoring is indicated. Some clinicians begin with a single, small test dose. Hepatic transaminases and urinary porphyrin output may rise transiently after institution of therapy, returning to normal levels as treatment continues. Dosing information listed in the full drug monograph for standard antimalarial or other nonporphyria indications is inappropriate for treatment of porphyria cutanea tarda.

It is available as a 200-mg tablet and is not available as a syrup. Crush tablet and mask the bitter taste in jam, applesauce, or other soft food.

Class Summary

In patients with anemia of chronic disease in whom venesections are relatively contraindicated, stimulation of erythropoiesis can mobilize tissue iron and may even enable low-volume phlebotomies to be performed at judicious intervals.

Epoetin alfa (Epogen, Procrit)

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Epoetin alfa stimulates the division and differentiation of committed erythroid progenitor cells. It induces the release of reticulocytes from bone marrow into the blood stream.

Class Summary

Iron chelators form complexes with tissue iron to excrete it from the body and remove the tissue iron from use.

Deferoxamine (Desferal)

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In addition to being an iron chelator, deferoxamine may reduce the compensatory increase of intestinal iron absorption often seen in remission induced by the greater degree of iron reduction achieved by phlebotomies.

Questions

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Media Gallery

Thickened skin with blisters, scars, and milia. Courtesy of Dirk Elston, MD.
Close-up image of blisters, scarring, and milia. Courtesy of Dirk Elston, MD.
Subepidermal bulla, festooning of rete ridges, hyalinization of blood vessel walls, solar elastosis, and caterpillar bodies. Courtesy of Dirk Elston, MD.
Fluorescence of urine with a Wood light examination. Courtesy of Brooke Army Medical Center Teaching File.

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Author

Anthony W Linfante, Jr, MD Resident Physician, Department of Dermatology, University of Texas Medical Branch School of Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Meredith M Gavin MD Candidate, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Meredith M Gavin is a member of the following medical societies: Alpha Omega Alpha, American Medical Association, American Medical Women's Association, Gold Humanism Honor Society, Texas Medical Association

Disclosure: Nothing to disclose.

Brandon Patrick Goodwin, MD Assistant Professor of Dermatology/Dermatopathology, Assistant Lab Director, Dermatopathology Laboratory, University of Texas Medical Branch School of Medicine; Ambulatory Medical Director, UTMB Health Dermatology Clinic

Brandon Patrick Goodwin, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society of Dermatopathology, Houston Dermatological Society, Texas Dermatological Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Julia R Nunley, MD Professor, Department of Dermatology, Virginia Commonwealth University Medical Center

Julia R Nunley, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American Society of Nephrology, International Society of Nephrology, Medical Dermatology Society, Medical Society of Virginia, National Kidney Foundation, Phi Beta Kappa, Women's Dermatologic Society

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: American Board of Dermatology<br/>Author for: Up-to-date.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Craig A Elmets, MD Professor and Chair, Department of Dermatology, Director, Chemoprevention Program Director, Comprehensive Cancer Center, UAB Skin Diseases Research Center, University of Alabama at Birmingham School of Medicine

Craig A Elmets, MD is a member of the following medical societies: American Academy of Dermatology, American Association of Immunologists, American College of Physicians, American Federation for Medical Research, Society for Investigative Dermatology

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: University of Alabama at Birmingham; University of Alabama Health Services Foundation<br/>Serve(d) as a speaker or a member of a speakers bureau for: Ferndale Laboratories<br/>Received research grant from: NIH, Veterans Administration, California Grape Assn<br/>Received consulting fee from Astellas for review panel membership; Received salary from Massachusetts Medical Society for employment; Received salary from UpToDate for employment. for: Astellas.

Maureen B Poh-Fitzpatrick, MD Professor Emerita of Dermatology and Special Lecturer, Columbia University College of Physicians and Surgeons

Maureen B Poh-Fitzpatrick, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, New York Academy of Medicine, New York Dermatological Society

Disclosure: Nothing to disclose.

Porphyria Cutanea Tarda Medication

Medication Summary

Antimalarials

Class Summary

Chloroquine (Aralen)

Chloroquine (Aralen)

Hydroxychloroquine (Plaquenil)

Hydroxychloroquine (Plaquenil)

Bone Marrow Stimulants

Class Summary

Epoetin alfa (Epogen, Procrit)

Epoetin alfa (Epogen, Procrit)

Chelators

Class Summary

Deferoxamine (Desferal)

Deferoxamine (Desferal)

Porphyria Cutanea Tarda Medication

Medication Summary

Antimalarials

Class Summary

Chloroquine (Aralen)

Chloroquine (Aralen)

Hydroxychloroquine (Plaquenil)

Hydroxychloroquine (Plaquenil)

Bone Marrow Stimulants

Class Summary

Epoetin alfa (Epogen, Procrit)

Epoetin alfa (Epogen, Procrit)

Chelators

Class Summary

Deferoxamine (Desferal)

Deferoxamine (Desferal)

References

Tables

Contributor Information and Disclosures

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