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Erythropoietic Protoporphyria- Symptoms, Causes, Treatment
What is Erythropoietic Protoporphyria?
Erythropoietic protoporphyria is a collection of genetic disorders known as porphyrias. This disease can occur due to loss in enzyme ferrochelatase which results in photosensitivity ie.damage of the skin due to light. It primarily affects to the upper surfaces of the hands and feet and to the face. Porphyrins is one of the chemical that plays an important role in the manufacturation of enzymes and blood within the body. Protoporphyrin may associates with bone marrow, red blood cells and sometimes the liver. It mostly found in white but can also found in people of any region.
Causes of Erythropoietic Protoporphyria
- Hot weather or sunlight is the main cause of this disease.
- Decrease in enzyme ferrochelatase is another reason for erythropoietic protoporphyria.
- Excess in the level of ethanol, alcohol and curtail carbohydrate may also cause of it.
What are the symptoms of Erythropoietic Protoporphyria?
- Painful photosensitivity is the main symptom of the erythropoietic protoporphyria
- Swelling, burning, itching, and redness of skin are the commom symptoms of erythropoietic protoporphyria.
- There may be also blistering and scarring can occurs on skin of the body.
- Damage of liver which leads to failure of liver, enlargement of the spleen and jaundice can also take place.
- In some cases skin may turn to thick and wrinkled.
Erythropoietic Protoporphyria Treatment
- There is no any specific cure for this disease.
- Use of sunscreen lotions containing zinc oxide or titanium dioxide and proper clothing is essential to protect from sunlight.
- Different types of doses like beta carotene, fexofenadine, cholestyramine, actidose are also useful in the treatment of erythropoietic protoporphyria.
- Use of drugs like tagamet, ursodiol, heminb and heme arginate for treatment is helpful.
- Liver transplantation treatment is use in some patients.
- Use of creams containing dihydroxyacetone or tanning gels which helps in the production of superficial pigmentation.
- Use of oral cysteine or pyridoxine to reduce the photosensitivity.
- Limited use of ethanol, alcohol and curtail carbohydrate is must in erythropoietic protoporphyria patient.
- Surgical treatment is also availablae for this disease.
- Narrowband UVB phototherapy is available which increases the percentage of melanin.
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