Erythropoietic Protoporphyria- Symptoms, Causes, Treatment and Home Remedies

What is Erythropoietic Protoporphyria?

Erythropoietic Protoporphyria is also known as Gunther's disease. Erythropoietic protoporphyria is a disease of porphyrin metabolism. Protoporphyrin accumulates in the bone marrow, red blood cells and sometimes the liver. Both males and females are equally affected. Erythropoietic Protoporphyria starts in childhood. This disease occur in young child having age of 11 years. It is due to an inherited deficiency of the enzyme ferrochelatase. It appears on the tops of the hands, feet, face and ears. People with EPP causes liver disease.

Causes of Erythropoietic Protoporphyria

• Abnormal elevated levels of protoporphyrin IX in erythrocytes , feces and plasma and by sensitivity to visible light. 
• Erythropoietic Protoporphyria is caused by inheritance.
• Reduction in activity of heme synthase to 10 to 25% of normal levels causes this disease.
• Erythropoietic Protoporphyria is caused by the production of porphyrins.
• Accumulation of protoporphyrins in the liver can cause liver damage.

What are the symptoms of Erythropoietic Protoporphyria?

• Swelling, burning, itching, and redness of the skin may appear in the patient.
• Discoloration of the skin
• Patient feels uncomfortable or painful burning sensation of the skin after sun exposure.
• The affected skin may become red and swollen and blistered.
• Malaise, pain under the ribs on the right, jaundice and increasing photosensitivity.
• Chronically sun-exposed skin may become thick and wrinkled.

Erythropoietic Protoporphyria Treatment

• Lumitene/Beta-carotene is the most effective therapy and the dosages is given to the patient according to age group.
• Protect the skin from exposure to operating lamps during a surgical procedure.
• Colestyramine reduces photosensitivity and hepatic protoporphyrin content.
• Antihistamines-these tablets or syrups may help the few people for whom the nettle rash.
• Phototherapy- They involve careful exposure to artificial ultraviolet light, usually three times a week for about five weeks in the spring, to allow the skin to thicken slightly and develop a tan.
• Blood transfusions and perhaps removing the spleen may reduce porphyrin production by the bone marrow.
• To avoid exposure to sunlight.
• A high-carbohydrate diet is typically recommended.

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