hormonal and metabolic disorders
Porphyria Cutanea Tarda
Introduction
Porphyria cutanea tarda is the most common Overview of Porphyrias and causes blistering and fragility of skin exposed to sunlight. People have chronically recurring blisters on the sun-exposed areas of their bodies. Excess iron can build up in the liver, causing liver damage. Doctors test urine and stool samples for high levels of porphyrins. Removing blood (phlebotomy), givingchloroquine(orhydroxychloroquine), or doing both is helpful. Porphyrias are a group of disorders caused by abnormalities of enzymes involved in the production of heme. Heme is a chemical compound that contains iron and gives blood its red color. Heme is a key component of several important proteins in the body. (See also Overview of Porphyrias .) Porphyria cutanea tarda occurs throughout the world. There are two main types: Type 1: Acquired or sporadic (in about 75 to 80% of people) Type 2: Hereditary or familial (in about 20 to 25% of people) As far as is known, the sporadic form of this porphyria is the only porphyria that can occur in people who do not have an inherited deficiency of an enzyme involved in heme production. Porphyria cutanea tarda results from underactivity of the enzyme uroporphyrinogen decarboxylase, which leads to accumulation of porphyrins in the liver. Liver disease is common. About 35% of people develop Cirrhosis of the Liver and 7 to 24% develop Porphyria cutanea tarda has several common precipitating factors. These factors include Excess iron in the liver Moderate or heavy alcohol use Smoking Takingestrogens Infection with Hepatitis C, Chronic None Hepatitis C infection is a major risk factor for development of porphyria cutanea tarda, and it should be looked for in all patients with this disorder. If present, the initial treatment of choice is direct-acting antiviral medications to cure hepatitis C virus infection, which will nearly always also lead to remission of porphyria cutanea tarda. Infection with the Human Immunodeficiency Virus (HIV) Infection (HIV) is a less common precipitating factor. These factors are thought to interact with iron and oxygen in the liver and thereby inhibit or damage the enzyme uroporphyrinogen decarboxylase. Some people receiving Dialysis for long periods develop a skin condition that resembles porphyria cutanea tarda because dialysis does not remove porphyrins well. This condition is termed porphyria cutanea tarda of end-stage renal disease. 