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Porphyria Cutanea Tarda



Porphyria Cutanea Tarda Causes


In individuals with PCT, familial, have inherited mutations of the UROD gene which produces an enzyme critical to the production of heme. The capacity of the enzyme is reduced to half than normal for persons with PCT, familial. Sporadic PCT can be induced or aggravated by excessive alcohol abuse due to the oxidative damage caused to the liver cells which in turn inhibits the activity of the UROD gene. Exposure to chlorinate cyclic hydrocarbons can also lead to a decreae activity of the UROD gene.


Porphyria Cutanea Tarda Definition


Porphyria cutanea tarda (PCT), is a disease characterized by having low levels of the enzyme used in producing heme, a molecule vital to all organs of the body. There are basically two types of PCT, the familial type and the sporadic type. The main difference between the two is that, as its name suggests, the familial type PCT is inherited.


Porphyria Cutanea Tarda Diagnosis


Primary diagnosis of PCT, familial can be based on the blisters and skin lesions that appear on the skin of individuals suffering from the disease. A more conclusive diagnosis of the disease, however, can only be made through laboratory testing. Persons wit PCT commonly have high levels uroporphyrinogen in their urine. It is also strongly suggested that testing for Hepatitis C and hemochromatosis be made since these conditions are closely related to PCT.


Porphyria Cutanea Tarda Symptoms and Signs


The symptoms of PCT include photosensitivity of the skin that results in blisters and erosions. People with the sporadic type of PCT experience liver problems such as hepatic fibrosis, cirrhosis and inflammation. Other symptoms of PCT include hyperpigmentation and hypertrichosis.


Porphyria Cutanea Tarda Treatment


Being a chronic condition, PCT familial can be treated best with proper management. Individuals suffering from PCT are strongly advised to avoid alcoholic beverages, iron supplements, excess exposure to sunlight, chlorinated cyclic hydrocarbons and estrogen.


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