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Porphyria Cutanea Tarda
WHAT YOU NEED TO KNOW:
What is porphyria cutanea tarda?
Porphyria cutanea tarda (PCT) is a disorder that causes skin to form blisters or lesions when exposed to sunlight. PCT is a form of porphyria, a disorder that affects how your body makes red blood cells (RBC). Your body needs a chemical called porphyrin to make heme, a part of RBC that carries oxygen. Porphyria prevents your body from creating enough enzymes to control the process, and porphyrin builds up. High levels of porphyrin can cause problems throughout your body, depending on where it builds up. PCT causes porphyrin to build up in your skin. Proteins are released that cause the skin to become overly sensitive to sunlight. This leads to the formation of skin lesions or blisters where the skin was exposed to sunlight. PCT can be genetic or caused by exposure to certain chemicals or infections.
What triggers a porphyria cutanea tarda attack?
- Cigarettes and alcohol
- Infections caused by viruses, such as hepatitis C and HIV
- Iron-related diseases, such as hemochromatosis, which causes too much iron to build up in body tissues
- Hemodialysis to filter the blood for long-term kidney failure
- Exposure to toxic chemicals
- Birth control pills and estrogen hormone therapy for treating prostate cancer
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 on exposed skin, 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.
How is porphyria cutanea tarda diagnosed?
Your caregiver will ask about your symptoms. He will ask about your medical history and if any family members have porphyria or other blood disorders. Samples of your blood, urine, or bowel movement may be collected. Blood tests check for the enzymes needed to control RBC production, and your porphyrin level.
How is porphyria cutanea tarda treated?
Caregivers may suggest that you avoid the conditions and substances that trigger your PCT. You may also need any of the following:
- Malaria medicine: Caregivers may give you certain medicines used to treat malaria. These antimalarial medicines remove excess porphyrins from the liver.
- Phlebotomy: This procedure is also called a venipuncture. For this procedure, blood is removed through your vein. This decreases the porphyrin levels in your liver and blood.
How can I prevent a porphyria cutaneous tarda attack?
- Do not smoke cigarettes or drink alcohol: Cigarettes and alcohol can damage the liver and further worsen your PCT. Talk to your caregiver if you have trouble quitting smoking or drinking.
- Be careful with medicine: Ask your caregiver if any of the medicines you are taking can trigger a PCT attack. Always check for skin changes when you take your medicines.
- Protect your skin: Keep your skin protected from sun and scratches. Wear lightweight, loose, and light-colored clothing. Protect your head and neck with a hat or umbrella when you are outdoors. Wear sunscreen that has a sun protectant factor (SPF) approved by your caregiver. Follow the directions on the label when you use sunscreen.
What are the risks of porphyria cutaneous tarda?
- Treatment for PCT may cause side effects. Medicines may cause nausea, vomiting, headache, itchiness, slow heartbeat, or seizures. Phlebotomy can cause your body to lose too much blood, and this may lead to anemia (low RBC levels). Your health, quality of life, and ability to function may decrease without treatment.
- Untreated porphyria cutanea tarda can cause more problems. You may have frequent skin damage from sun and small injuries. This may cause skin ulcers, infections, bleeding, and swelling. You can develop cirrhosis or liver cancer.
When should I contact my caregiver?
Contact your caregiver if:
- You have a fever.
- You have chills or a cough, or feel weak and achy.
- You have pain, redness, or swelling around your skin blisters.
- Your skin blisters have a bad smell or pus coming out of them.
When should I seek immediate help?
Seek help immediately or call 911 if:
- You have a fast heartbeat or chest pain.
- You have trouble breathing all of a sudden.
Care AgreementYou have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your caregivers to decide what care you want to receive. You always have the right to refuse treatment. The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.
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