Porphyria Cutanea Tarda
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GENERAL INFORMATION:
What is porphyria cutanea tarda?
- Porphyria cutanea tarda, also called PCT, is a condition which affects the skin when it is exposed to light. PCT is the most common form of porphyria. Porphyria is an inherited blood disorder where the body cannot break down certain chemicals. Normally, the liver makes many enzymes (chemicals) that break down substances, such as porphyrins and iron. Porphyrins are broken down by the enzyme uroporphyrinogen decarboxylase (UROD) to form the pigment heme. Heme gives blood its red color and helps blood to carry oxygen.
- With PCT, the enzyme UROD may not work correctly, or may be absent or present only in small amounts. Porphyrins and iron build up in the body making the skin overly reactive to light. When exposed to light, proteins are released that cause damage and inflammation (swelling) to the skin. This leads to the formation of many skin lesions or blisters (fluid-filled bumps).
What causes porphyria cutanea tarda? Porphyrias are caused by a defect in the gene that makes the enzyme UROD. A gene contains the information needed to make a certain substance in the body. Porphyria cutanea tarda is either inherited (passed from parents to their children) or acquired (developing after birth).
- Inherited: This type appears in childhood, but often no symptoms are present.
- Acquired: This type is more common, and symptoms usually appear in adults. The following conditions may cause or trigger a PCT attack:
- Cigarette smoking and drinking alcohol too much and too often. Alcohol is found in beer, wine, liquor (vodka and whiskey), and other adult drinks.
- Infections caused by viruses (germs), such as hepatitis C and human immunodeficiency virus (HIV).
- Iron-related diseases, such as hemochromatosis, which cause too much iron in body tissues.
- Hemodialysis for long-term kidney failure. Hemodialysis uses a machine to clean or filter blood when the kidneys are failing.
- Too much exposure to toxic chemicals such as chlorinated hydrocarbons.
- Using birth control pills and estrogen hormone therapy for treating prostate cancer.
- Cigarette smoking and drinking alcohol too much and too often. Alcohol is found in beer, wine, liquor (vodka and whiskey), and other adult drinks.
What are the signs and symptoms of porphyria cutanea tarda? The signs and symptoms are mostly confined to the skin and are worse during summer. Blisters usually form on the sun-exposed areas, such as the arms, face, and backs of the hands. These blisters usually become crusted, scarred, and take a long time to heal. Your skin may also be very delicate and easily damaged by small injuries, such as scratches or burns. Your skin color may either be too light or too dark. You may also have increased facial hair or no hair growth on your scalp. Other symptoms may also be present if your liver becomes affected.
How is porphyria cutanea tarda diagnosed? Your caregiver will take your past and present health history. He may want to know the details of your skin problems. Your caregiver may also need to know if other family members have porphyria or other blood disorders. You may also need any of the following:
- Blood, urine, or stool tests: Samples of your blood, urine, or stool are collected and sent to a lab for tests. These will check the levels of your porphyrins. Caregivers may also learn more about your blood with these tests.
- Skin biopsy: Your caregiver may need to remove a small sample of your skin. This skin tissue will then be sent to a lab for different tests. Ask your caregiver for more information if you need a skin biopsy.
How is porphyria cutanea tarda treated? Caregivers may suggest that you avoid the conditions and substances that trigger your PCT. Correct skin care is also very important. You may also need any of the following:
- Medicines: Caregivers may give you certain medicines used to treat malaria. These anti-malarial medicines remove excess porphyrins from the liver. Ask your caregiver for more information about anti-malarials as treatment for porphyria.
- Phlebotomy: This procedure is also called a venipuncture. For this procedure, a certain amount of blood is removed through your vein. With phlebotomy, the excess iron is slowly removed to make the enzyme UROD work properly. This decreases the porphyrin levels in your liver and blood.
How can I prevent attacks of porphyria cutaneous tarda? The following may help you decrease your chances of triggering PCT:
- Avoid smoking cigarettes and drinking alcohol. Smoking and drinking alcohol increase the chances of making your PCT worse. They may also damage the liver and further worsen your problems. Talk to your caregiver if you have trouble quitting smoking or drinking alcohol.
- Be careful with the medicines you are taking. Some medicines, including over-the-counter or prescribed medicines, may trigger a PCT attack. Ask your caregiver before using or taking any medicines, such as those that contain the estrogen hormone. Always check for skin changes when taking your medicines.
- Cover your skin. Keep your skin protected by wearing clothing to cover it. Wear lightweight, loose, and light-colored clothing. Protect your head and neck with a hat or umbrella when you are outdoors.
- Protect your skin from too much sun exposure. Wear sunscreen that has a sun protectant factor (SPF) approved by your caregiver. Follow the directions on the label when using sunscreen.
Where do I get support and more information? Having PCT may be life-changing for you and your family. Accepting that you have PCT may be hard. You and those close to you may feel sad or frightened. These are normal feelings. Talk to your caregivers, family, or friends about your feelings. You may also want to join a porphyria support group. This is a group of people who have porphyria. Ask your caregiver for the names and numbers of support groups. Contact the following for more information:
- American Porphyria Foundation
4900 Woodway, Suite 780
Houston, TX 77056
Phone: 1-713-266
Web Address: http://www.porphyriafoundation.com
CARE AGREEMENT:
You have the right to help plan your care. To help with this plan, you must learn about your health condition and how it may be treated. You can then discuss treatment options with your caregivers. Work with them to decide what care may be used to treat you. You always have the right to refuse treatment.
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