Hepatitis C and Porphyria Cutanea Tarda (PCT)
Porphyrins are complex molecules in the body that combine with iron to produce heme, which is responsible for giving blood its red color. Porphyrins combine with globin to form hemoglobin. Porphyria is the name of a group of diseases caused by an excess of porphyrins in the blood. Porphyria Cutanea Tarda (PCT) is the most common type of porphyria, triggered by a deficiency of an enzyme called uroporphyrinogen decarboxylase (UROD for short). The reduced activity of UROD leads to an overproduction and buildup of the protein uroporphyrinogen in the blood and urine of patients. This results in an abnormal production of heme, a compound found in all body tissues and especially in the liver, bone marrow, and red blood cells.
PCT has a strong association with infection of hepatitis C.1 Therefore, we should test, treat and cure people with hepatitis C as early as possible to prevent or lessen the effect of PCT.
Note: Most of the medications to treat hepatitis C are expensive, and many insurance companies and Medicaid/ Medicare are restricting HCV treatment to people with the most severe cases. One of the conditions that may qualify people for HCV treatment is an extrahepatic manifestation such as PCT. Discuss any symptoms with your medical provider and have symptoms recorded in your medical records. If you are not approved for the drugs to treat hepatitis C, you may qualify for free medications or co-pay assistance through a pharmaceutical patient assistance program or other assistance programs. More information is available at the end of this article.
More Information – PCT
PCT can be caused or triggered by hemochromatosis (accumulation of iron in the liver), heavy alcohol consumption, estrogens (oral contraceptives, and prostate cancer treatment), viral infections (hepatitis C and HIV), and possibly smoking. Hepatitis C is the most common infection associated with viral infections that may account for up to 50% of PCT infections.
Symptoms: The symptoms of PCT are mostly confined to the skin. Skin lesions or blisters are most often seen on the hands, forearms, back of the neck and face and areas exposed to the sun. The skin may become red, blister and peel after exposure to the sun or minor trauma. PCT can also cause either darkening or lightening of the skin, increased facial hair, scarring, alopecia (hair loss), thickening of the skin, itching and premature aging of the skin. In severe cases, calcium may be deposited in the skin causing non-healing ulcers. Liver function enzymes can be abnormal although enzymes are usually only mildly elevated. A liver biopsy is generally performed to assess iron stores and to check for any damage caused by PCT. PCT is usually diagnosed by a blood test that measures porphyrins in the blood, and the diagnosis is confirmed by testing for porphyrins in the urine and feces.
Management: PCT cannot be cured, but it is one of the easiest of the porphyria conditions to manage and treat. The most common management methods include:
- Phlebotomies (the removal of blood) to reduce iron in the liver. Usually, about 5-6 pints of blood are removed—this consists of 1 pint of blood every one or two weeks. Phlebotomies are given until the level of serum ferritin (iron) is reduced to about 20ng/ml. Phlebotomies can also reduce the porphyrins to normal levels in the blood. Once levels of ferritin and porphyrins have normalized PCT does not usually recur.
- Avoid the sun when possible, use sunscreen and protective clothing such as gloves, hats, pants, and long-sleeved shirts when outside.
- Low doses of chloroquine or hydroxychloroquine (drugs that are used to treat malaria).
- Restrict foods that contain iron.
- Treatment of the underlying disease (HCV) has also been found to decrease skin lesions as well as the amount of UROD’s found in urine.
According to the Porphyria Foundation “The treatment of PCT is almost always successful, and the prognosis is usually excellent.” That is –make sure to find a medical provider (s) who is well-versed in PCT and hepatitis C. Also, be sure to find one who will treat and cure hepatitis C.
1 American Association for the Study of Liver Disease
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