Porphyria Cutanea Tarda

Definition

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 are blisters which become ulcerated in areas of the skin exposed to sunlight, especially on the face, ears and dorsum 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. Porphyria Cutanea Tarda comes in two forms which are :

• The sporadic form of porphyria cutanea tarda :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.
• The familial form of porphyria cutanea tarda : It affected in multiple generations. The enzyme UROD is reduced in all tissues.

Causes of Porphyria Cutanea Tarda

Some causes of porphyria cutanea tarda are as:-

• 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.
• 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.

Symptoms of Porphyria Cutanea Tarda

The possible symptoms of porphyria cutanea tarda are :-

• Liver function abnormalities are common but are usually mild. Porphyria Cutanea Tarda is often associated with hepatitis C infection, which also can cause these liver complications.
• The increased hair growth is the primary symptom.
• 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.
• Darkening and thickening of the skin may also occur.

Treatments of Porphyria Cutanea Tarda

The effective treatments for porphyria cutanea tarda are as:-

• 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 Porphyria Cutanea Tarda should not take any medicine without consulting their doctor.