Porphyria Cutanea tarda

DEFINITIONS

Porfiria Cutanea form porfiria tarda is the most frequently found, which causes blisters on the skin exposed to sunlight.

CAUSE

Tarda Porfiria cutanea happening around the world and is the only form that is not porfiria hereditary (hereditary disease).

This disease is a hepatic porfiria, occurs when uroporfirinogen decarboxylase (one on the liver enzyme essential for the formation of heme), becomes inactive.
Factors proponent of this disease are:
- Iron
- Alcohol
- Estrogen
- Hepatitis C virus infection

Sometimes porfiria cutanea tarda occurs in patients infected by HIV.

Although this disease is not inherited, sometimes uroporfirinogen decarboxylase enzyme deficiency that is partial, inherited by one of his parents and makes a person easy to suffer from this disease.
Such cases are called familial porfiria kutanea tarda.
SYMPTOMS

Skin blisters occur in areas exposed to sunlight, like the back of the hands, arms and face.
The skin, especially skin of the hands, is also very sensitive to trauma.

Skin blisters will be followed by scab formation and scar tissue, which takes a long time for the healing process.

Skin damage occurs due to porphyrin produced in the liver transmitted by blood plasma into the skin.

An increase in facial hair growth.

Liver is usually slightly damaged due to hepatitis C virus infection or due to excessive alcohol consumption.
After a while, can occur even cirrhosis of the liver and liver cancer.

Diagnosis

To make a diagnosis porfiria cutanea tarda, examination of blood plasma, urine and stool to the possibility of porphyrin.
Porfiria which causes lesions (wounds) on the skin accompanied by high levels of porphyrin in the blood plasma.
In porfiria kutanea tarda, porphyrin levels in urine and feces also increases.

TREATMENT

Porfiria cutanea the porfiria tarda is the most easily treated.
Performed a procedure called phlebotomi, where approximately 0.5 L of blood was taken every 1-2 weeks.
Phlebotomi will make the patient have iron deficiency mild.

Porphyrin levels in the liver and blood plasma will fall in stages, the skin will improve and eventually become normal again.
Phlebotomi usually done only 5-6 times; anemia occurs when too often done phlebotomi.

If the disease recurs, it may be necessary [extra hlebotomi.

Klorokuin or hidroklorokuin in small doses is also effective.
The drugs are out excess porphyrin from the liver.
But high doses cause telalu porphyrin spending too fast, so for a while can make things worse pofiria kutanea tarda and liver damage.

Avoiding alcohol can help speed healing.