What causes porphyria cutanea tarda?
Who gets and what is the cause of porphyria cutanea tarda? PCT is caused by a defect in a liver enzyme uroporphyrinogen decarboxylase (UROD). It is hereditary in one-third of patients when there is usually a family history of the condition and symptoms begin in early adult life.
Why is porphyria called the vampire disease?
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.
What causes porphyria?
These disorders are usually inherited, meaning they are caused by gene mutations link passed from parents to children. If you have porphyria, cells fail to change chemicals in your body—called porphyrins and porphyrin precursors—into heme, the substance that gives blood its red color.
What does porphyria do to the skin?
Cutaneous porphyrias Sensitivity to the sun and sometimes artificial light, causing burning pain. Sudden painful skin redness (erythema) and swelling (edema. Blisters on exposed skin, usually the hands, arms and face. Fragile thin skin with changes in skin color (pigment)
Is porphyria cutanea tarda curable?
PCT is the most treatable form of porphyria and treatment appears equally effective for both the sporadic and familial forms. The standard treatment of individuals with PCT is regularly scheduled phlebotomies to reduce iron and porphyrin levels in the liver.
Can you live a normal life with porphyria?
Patients with porphyria generally have a normal life expectancy. However, those with acute hepatic porphyria are at increased risk of developing high blood pressure, chronic kidney disease, and hepatocellular carcinoma (liver cancer), which may reduce their lifespan.
What food causes porphyria?
There is no evidence that food or drink will trigger acute porphyria symptoms, except for alcohol. As with any illness or discomfort, don’t assume (or let your doctor assume) that anything new is always due to the porphyria.
What foods to avoid if you have porphyria?
People with porphyria are advised to maintain a diet with an average or higher-than-average intake of carbohydrates, which can lessen disease activity—but they are also advised to avoid refined sugars, corn syrup and heavily processed foods.