What is porphyria cutanea Tarda?
Porphyria cutanea Tarda is the most common type of group of enzyme deficiencies classified as porphyria. This is caused by insufficient levels of uroporphyrinogen III decarboxylase or urod, which encodes the fifth enzyme required in the production of HEMU, important molecules in human physiology. HEME is part of the hemoglobin molecule that transports oxygen in the blood. Porphyria Cutanea Tarda is characterized by sensitivity to sunlight, causing the skin to blister when it is exposed to too much sun.
Although porphyria cutanea Tarda occurs more often than other types of porphyria, it is very rare and affects only one in 25,000 people. About 80% of cases are sporadic or inherited. In addition, the disorder is often asymptomatic.
blisters caused by photosensitivity in patients with this disease most often affect the face, lower legs and forearm. Heals slowly and often a scar. In some patients, photosensitivity is manifested as NelIBIERS, but as hyperpigmentation or darkening of the skin or as hypertrichosis, abnormal hair growth, often on the face. In patients who have not inherited the disease, the inflammation and scarring of the liver is common.
sporadic porphyria cutanea Tarda differs from inherited disease in that the gene of urris shows no mutations. The body has an abnormally high demand for heme and also enzymes involved in the production of heme. Risk factors for porphyria cutanea Tarda include alcoholism, excessive iron or estrogen, infection of hepatitis C, cancer and mutation of protein hemochromatosis or HFE gene. These risk factors can also deepen the disease in people with a inherited Urod gene.
Inherited or familial porphyria cutanea Tarda is sometimes referred to as type I, while sporadic variety is classified as type II. The most precious subtype, type III, shows the family pattern in that more than one cell is influencedEN families, but does not include the mutation of the Urod gene. In this case, the HFE gene mutation can be a genetic factor.
Porphyria cutanea Tarda is usually diagnosed with a urine test that reveals high levels of uroporphyrinogen. If laboratory tests do not reveal any signs of the disease, but the patient experiences his symptoms, diagnosis is often pseudoporphyria. This condition is not well understood, but may be due to an allergic reaction to drugs or excessive exposure to ultraviolet rays with long waves (UVA), as in a solar stand.
Porphyria cutanea Tarda is a chronic condition without a known drug, so the treatment is aimed at alleviating symptoms. Patients are recommended to avoid alcohol, food rich, sunlight and estrogen. Treatment may also include treatment of hepatitis C as needed, bloodshed to reduce iron in the body and chlorochin drugs, also used to treat and prevent malaria.