The disorder results from low levels of the enzyme responsible for the fifth step in heme production. 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. PCT is essentially an acquired disease, but some individuals have a genetic (autosomal dominant) deficiency of UROD.
This type of porphyria occurs in an estimated 1 in 25,000 people, including both inherited and sporadic (noninherited) cases. It also makes the skin sensitive to minor trauma. Crusting and scarring can also occur and will take a long time to cure.This skin damage happens because of the metabolite porphyrin. Blisters develop on sun-exposed areas of the skin, such as the hands and face.
The skin in these areas may blister or peel after minor trauma. Increased hair growth, as well as darkening and thickening of the skin may also occur. Neurological and abdominal symptoms are not characteristic of PCT. In about 80% of people with porphyria cutanea tarda, the disorder does not appear to be hereditary and is called sporadic. In the remaining 20%, the disorder is hereditary and is called familial.
An estimated 80 % of porphyria cutanea tarda cases are sporadic. The exact frequency is not clear because many people with the condition never experience symptoms. PCT is the most treatable of the porphyrias. Treatment seems to be equally effective in familial and non-familial PCT. Antimalarial tablets i.e low-dose chloroquine or hydroxychloroquine may be recommended, but must be used cautiously.
Chemicals or medicines that are toxic to the liver or are known to be capable of aggravating the condition, including iron supplements, should be avoided. Alcohol intake should be reduced or, preferably, stopped completely. Successful antiviral therapy of chronic hepatitis C may also produce remission, as may low-dose therapy with anti-malarial drugs such as chloroquine.
Porphyria Cutanea Tarda Treatment and Prevention Tips
1. Avoid alcohol, oestrogen, and iron.
2. Apply an opaque sun-block and cover up when outside.
3. Use tanning cream containing dihydroxyacetone.
4. Chloroquine and venesection can be part of a management strategy.
5. Antimalarial tablets i.e low-dose chloroquine or hydroxychloroquine may be recommended.
6. Wash hands immediately after application to avoid tanned palms. Alternatively, wear gloves to apply.
7. To avoid staining of clothes, wait 30 minutes for the product to dry before putting on clothes.
8. Don't shave, bathe, or swim for at least an hour after applying the product.
Posted in: Porphyria Cutanea Tarda
Related Post:
Widget by [ Iptek-4u ]
