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Porphyria Cutanea Tarda Information and Treatment

Porphyria cutanea tarda is very common iron deficiency and overload disorders. The most common porphyrias are porphyria cutanea tarda, acute intermittent porphyria , and eryhtropoietic protoporphyria . In porphyria cutanea tarda (PCT) this enzyme defect may be within the liver and, in some people, also in the red blood cells. Some people are born with the condition, but in the majority it appears later in life, having been caused by a toxin (such as some pesticides and fungicides) affecting the liver. Porphyria can affect children at an early age, and at puberty.  Young and middle-aged adults may also suffer from this condition. When signs and symptoms occur, they usually begin in adulthood and result from the skin becoming overly sensitive to sunlight. Areas of skin exposed to the sun develop severe blistering, scarring, changes in pigmentation, and increased hair growth. Exposed skin becomes fragile and is easily damaged. People with porphyria cutanea tarda also have increased iron levels in the liver. They face a higher risk of developing abnormal liver function and liver cancer. The signs and symptoms of this condition are triggered by nongenetic factors such as alcohol abuse, excess iron, certain hormones, and viral infections.

Porphyria cutanea tarda is the most common subtype of porphyria . Cases are sporadic or hereditary. The disease is associated with ingestion of certain medications (eg, estrogens), and liver disease from alcoholism or hepatitis C. In patients with liver disease, hemosiderosis is often present. Heme is a vital molecule for all of the body's organs. It is a component of hemoglobin ,the molecule that carries oxygen in the blood. Affected individuals develop fragile skin, sores (erosions), blisters (vesicles and bullae), and tiny cysts (milia) on the sun-exposed areas i.e. the back of the hands and the forearms. They may notice that they sunburn easily. Some people develop mottled brown patches around the eyes and increased facial hair. Occasionally the skin becomes hardened (sclerodermoid) on the neck, face or chest. There may be small areas of permanent baldness (alopecia) or ulcers.

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