Porphyria Cutanea Tarda - Causes, Symptoms and Treatment
Porphyria cutanea tarda is a light-sensitive skin disease resulting in bullae that rupture easily to form shallow ulcers. The hallmarks of porphyria cutanea tarda (PCT) are blisters which become ulcerated in areas of the skin exposed to sunlight, especially on the face, ears and dorsum (back) of the hands.
It is the most common type of porphyria. It is a disorders of certain enzymes in the heme biosynthetic pathway or porphyrin pathway. 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 comes in two forms which are :
- The familial form of PCT : It affected in multiple generations. The enzyme UROD is reduced in all tissues.
- The sporadic form of PCT :is more common. The enzyme UROD is only reduced in the liver. It appears sporadically in people with liver disease, as from alcoholism and from exposure to agents such as estrogens.
Causes of Porphyria Cutanea Tarda:
Some causes of porphyria cutanea tarda are :-
- Genetics: The heredity problem is the main cause of the porphyria cutanea tarda. The activity of enzyme is usually reduced by 50 % in all tissues in people with the inherited form of the condition.
- Excess alcohol intake, may also lead to porphyria cutanea tarda.
- The excess iron can increase the demand for heme and the enzymes required to make heme. It reduces the activity of uroporphyrinogen and disrupts heme production and allows by products of the process to accumulate in the body, triggering the signs and symptoms of porphyria cutanea tarda.
- Estrogens and infection with hepatitis C or HIV can also trigger this disease.
Symptoms of Porphyria Cutanea Tarda:
The possible symptoms of porphyria cutanea tarda are :-
- The increased hair growth is the primary symptom.
- Darkening and thickening of the skin may also occur.
- Liver function abnormalities are common but are usually mild. PCT is often associated with hepatitis C infection, which also can cause these liver complications.
- People with porphyria cutanea tarda experience chronic, recurring blisters of various sizes on sun-exposed areas such as the arms, face, and especially the backs of the hands.
Treatments of Porphyria Cutanea Tarda:
The effective treatments for porphyria cutanea tarda are :-
- Phlebotomy is the primary symptom in which a pint of blood is removed. With phlebotomy, the excess iron is gradually removed, the activity of uroporphyrinogen decarboxylase in the liver returns toward normal.
- Avoid alcohol and other precipitating factors which are general cause of this disease..
- ARALEN and PLAQUENIL are also effective in treating porphyria cutanea tarda. These drugs remove excess porphyrins from the liver.
- The technique known as venesection, help in reducing the body's iron stores.
- Anyone with PCT should not take any medicine without consulting their doctor.