The terms porphyrin and porphyria are derived from the Greek word porphyrus, it means purple. Porphyria is the name given a group of very rare metabolic disorders that can occur when your body is unable to produce enough of a substance which is called haem. It can be appear in childhood but usually seen between the ages of 20 and 40. It affects women more than men.
Porphyrins are essential for the function of hemoglobin. A protein in your red blood cells that links to prophyrin, binds iron and carries oxygen to your organs and tissue. Porphyria disease mainly affects your nervous system, skin and other organs. There is a complex process that goes on in the liver and in red blood cells to make haem. The process has many steps and every step is controlled by a special protein which is called enzyme. If you inherit or develop a defect in these enzymes, the process may not work properly and can cause porphyrins in your body.
Porphyria Cutanea Tarda:
Porphyria cutanea tarda isdeficiency of the enzyme uroporphyrinogen decarboxylase. Manycutaneous blisters develop on the sun exposed areas of the skin like hands and face. These areas may blisters or peel after trauma. Porphyrin levels are extremely high in most cases of congenital porphyria and the more severe blistering skin lesions often lead to infection and mutilation with loss of facial features and fingers. It has several precipitating factors. These factors are include excess iron in the liver, heavy alcohol use. Infections with hepatitis C virus and smoking. When your skin exposed to sunlight, redness and many blisters are appear your skin may be itchy, and there may be areas where it is hyper pigmented or excessively hairy. Infection with HIV is a less common factor. These factor are thought to interact with iron and oxygen in the liver and thereby or damage the enzyme uroporphyringen decarboxylase.
Acute intermittent Porphyria:
Acute intermittent porphyria is the commonest type. In this disease only acute attacks and the skin never affected. Acute attacks always start with severe pain which is usually in the abdomen but nay also be felt the back or thighs.it is characterized clinically by life threatening acute neurovisceral attacks of severe abdominal pain without peritoneal signs,Hypertension, Vomiting, nausea and constipation are common. Some people become very confused during an acute attack and late find it difficult to remember details of their illness. The specific enzyme defect in each type of acute porphyria is the end result of an alternation in a hereditary particle of DNA known as gene. This is one of the hereditary hepatic porphyrias. Its inheritance is autosomal dominant.
There are some porphyria symptoms of are Discuss below
- Light sensitivity
- Abdominal pain
- Sudden attacks
- Heat sensations
- Agitation , confusion and seizures
- Mental disorders
- Personality changes
- Skin scarring
- Low blood pressure
- Muscle weakness
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