Overview
Porphyria cutanea tarda (PCT) is a type of porphyria or blood disorder that affects the skin. PCT is one of the most common types of porphyria. It’s sometimes referred to colloquially as vampire disease. That’s because people with this condition often experience symptoms following exposure to sunlight.
Most of the symptoms of porphyria cutanea tarda appear on the skin. Common symptoms include:
- blisters on skin that’s exposed to the sun, including the hands, face, and arms
- photosensitivity, which means your skin is sensitive to the sun
- thin or fragile skin
- increased hair growth, usually on the face
- crusting and scarring of the skin
- redness, swelling, or itching of the skin
- sores developing after minor injuries to the skin
- hyperpigmentation, which means patches of skin become darker
- urine that is darker than normal or reddish brown
- liver damage
After the blisters form on your skin, the skin may peel. It’s also common for scarring to appear once the blisters heal.
Hyperpigmentation patches usually appear on the face, hands, and neck.
Porphyria cutanea tarda may be caused by various things. The causes are usually categorized as either genetic or acquired.
The most common genetic causes include:
- family history of porphyria cutanea tarda
- inherited deficiency of the liver enzyme uroporphyrinogen decarboxylase
- family history of liver disease or liver cancer
- more liver iron than normal
The most common acquired causes include:
- alcohol consumption
- using estrogen therapy
- using oral contraceptives
- exposure to certain environmental factors or chemicals, such as Agent Orange
- taking too much iron
- smoking
- having hepatitis C
- having HIV
In some cases, it’s not possible to determine the cause of porphyria cutanea tarda.
You’re at a higher risk of porphyria cutanea tarda if you smoke or use alcohol. You’re also more likely to get this condition if you have hepatitis C or HIV.
Being exposed to certain chemicals, such as Agent Orange, can also increase your risk. You may have been exposed to this chemical if you’re a veteran who served in an area that had Agent Orange.
Porphyria cutanea tarda can affect both men and women. It usually appears after the age of 30, so it’s not common among children or teens.
Porphyria cutanea tarda affects people around the world and isn’t limited to a particular region or country. It’s estimated that 1 out of 10,000 to 25,000 people has this condition.
Your doctor may do a physical exam, check for symptoms, and record your medical history. In addition, they may use the following tests to diagnose porphyria cutanea tarda:
- blood tests
- urine tests
- stool tests
- skin biopsy
The doctor will check your levels of porphyrin and liver enzymes. Genetic testing may be recommended for people with a family history of this condition.
The treatment for porphyria cutanea tarda focuses on managing and stopping the symptoms. Lifestyle changes, such as limiting alcohol consumption and not smoking, may also help.
Common treatment options include:
- phlebotomy, which is the removal of blood to reduce iron
- chloroquine (Aralen)
- hydroxychloroquine (Plaquenil)
- pain medications
- iron chelators
- treating the diseases that cause the porphyria cutanea tarda, such as HCV or HIV
Phlebotomy is one of the most common treatments for porphyria cutanea tarda. Antimalarial tablets are also frequently used.
Common lifestyle changes to treat porphyria cutanea tarda include:
- avoiding alcohol
- not smoking
- avoiding sunlight
- using sunscreen
- avoiding injuries to the skin
- not taking estrogens
You may have to wear sunscreen, long sleeves, and a hat to avoid the sun.
Porphyria cutanea tarda can increase the risk of liver cancer or cirrhosis, which is scarring of the liver. This is why it’s important not to drink alcohol if you have this condition.
Porphyria cutanea tarda usually affects adults who are older than 30. It’s a blood disorder that mostly affects the skin. Your skin may be more sensitive to the sun, so you may need to take extra precautions to avoid the sun. Blisters are common from this condition.
Your doctor may recommend different treatments for porphyria cutanea tarda. Phlebotomy and antimalarial tablets are the most common treatment options.