2009-11-16 15:25:31 -

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Porphyria is a rare disorder characterized by excessive formation and excretion of porphyrins. These chemical compounds are normally utilized in heme production for hemoglobin. The disorders are classified as either erythropoietic or hepatic, depending of the site of overproduction and accumulation. These disorders can be inherited or acquired due to a genetic mutation.
Porphyria cutaneous tarda is the most common type of porphyria. It results from diminished enzymatic activity due to mutations. It can be an acquired disorder or may be inherited. Both forms lead to increased levels of iron in the liver.

Causes of Porphyria Cutaneous Tarda


This group of disorders is caused by either an inherited or acquired disorder involving enzymes of the heme pathway. This enzymatic pathway is primarily involved

with producing heme for the hemoglobin in red cells. It also produces heme in the liver.
Excess production of porphyrins causes them to be circulated in the blood stream. They diffuse into the skin, where they react with sunlight producing cutaneous photosensitivity.

Signs and Symptoms of Porphyria Cutaneous Tarda


Porphyria cutaneous tarda results in the development of chronic photosensitivity. The exposed skin, commonly on the face, neck and backs of the hands, becomes fragile. Vesicles develop which may rupture. They are slow to heal. Areas of damaged skin may develop increased growth of hair and often appears with darker pigmentation than the normal skin. The skin may also become scared and indurated.

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