Porphyria Cutanea Tarda Treatment & Management

  • Author: Maureen B Poh-Fitzpatrick, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: May 28, 2010
 

Medical Care

Sunlight avoidance is the main defense for photosensitivity until clinical remission can be induced. Alcohol must be proscribed. Cessation of tobacco smoking should be recommended, not only for potential beneficial effect on porphyria, but for overall good health. Estrogen use should be discontinued unless its need outweighs its adverse effects on porphyrin metabolism. After achievement of remission, estrogen therapies may be cautiously reinstituted; however, the duration of remissions may be shortened. Remissions may last from several months to many years. If symptoms recur, re-treatment can restore remissions.

Therapeutic phlebotomy[41, 42, 43] reduces iron stores, which improves heme synthesis disturbed by ferro-mediated inhibition of uroporphyrinogen decarboxylase (UROD). The goal of therapy is to reduce serum ferritin levels to the lower limit of the reference range.[44] Venesections may be scheduled at intervals ranging from a unit of whole blood removed twice weekly to every 2-3 weeks as tolerated by the patient. Care should be taken to not induce anemia (hemoglobin < 10-11 g/dL). Phlebotomy is the preferred therapy for individuals with a heavy iron burden. Efficacy of antihepatitis C therapy appears to be enhanced if hepatic siderosis is first reduced by phlebotomy.[45]

For patients in whom phlebotomy is not convenient or is contraindicated or for patients with relatively mild iron overload, oral chloroquine phosphate (125-250 mg PO twice weekly) or hydroxychloroquine sulfate (100-200 mg PO 2-3 times/wk), doses much lower than those used for antimalarial or photoprotective indications, can be effective.[46, 47, 48, 49, 50, 51] 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. Low-dose chloroquine and phlebotomy therapies may be used concomitantly to more rapidly reach clinical and biochemical remissions.[52]

Chelation with desferrioxamine is an alternative means of iron mobilization when venesections are not practical.[53]

For patients with porphyria cutanea tarda who are anemic from other chronic diseases (eg, renal failure, human immunodeficiency viral infection), human recombinant erythropoietin can be used to stimulate erythropoiesis.[54] This mobilizes tissue iron and may increase the circulating erythrocyte mass to a degree that permits therapeutic phlebotomies to be performed at judicious volumes and intervals.

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Consultations

Consultation with a gastroenterologist or a hepatologist for evaluation and treatment of viral hepatitis, liver damage due to alcohol abuse or hemochromatosis, and hepatic tumors may be warranted.

Consultation with a hematologist may be helpful in cases of suspected hemochromatosis or for management of phlebotomy or iron chelation therapies.

Consultation with a gynecologist for alternative forms of treatment for female patients in whom therapeutic use of estrogenic hormones is a probable inducing factor is often helpful.

For male patients treated with estrogen for prostatic carcinoma, consultation with the treating oncologist regarding the need for continued therapy is indicated.

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Diet

Iron-rich foods should be consumed in moderation; strict avoidance is not usually necessary. Adequate levels of vitamin C may retard oxidation reactions in the liver. Daily intake of fresh fruits and vegetables, their juices, or a vitamin supplement should be recommended as part of a healthy diet.

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Activity

Patients should avoid sunlight exposure until biochemical and clinical remission has been induced. Manual labor should be curtailed to minimize the mechanical trauma that causes erosions and blistering.

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Contributor Information and Disclosures
Author

Maureen B Poh-Fitzpatrick, MD  Professor Emerita of Dermatology and Special Lecturer, Columbia University College of Physicians and Surgeons; Professor of Medicine (Dermatology), University of Tennessee Health Science Center College of Medicine

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

Disclosure: Lundbeck, Inc. Honoraria Review panel membership; Clinuvel Pharmaceuticals, Ltd. Honoraria Consulting

Specialty Editor Board

Craig A Elmets, MD  Professor and Chair, Department of Dermatology, Director, 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, and Society for Investigative Dermatology

Disclosure: Palomar Medical Technologies Stock None; Amgen Consulting fee Review panel membership; Astellas Consulting fee Review panel membership; Massachusetts Medical Society Salary Employment; Abbott Laboratories Grant/research funds Independent contractor

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University 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, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Julia R Nunley, MD  Professor, Program Director, Dermatology Residency, 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, and Women's Dermatologic Society

Disclosure: Novartis Grant/research funds Consulting; Biolex Grant/research funds sub-investigator

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

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

Disclosure: Nothing to disclose.

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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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