Complete Information on Erythropoietic Protoporphyria

Erythropoietic protoporphyria (EPP) is a rare disorder dominating autosomal. Protoporphyria d' Erythropoietic (EPPalso called is the disease and the porphyrias gunther . the protoporphyria of Erythropoietic is a relatively soft form of porphyria. Erythropoietic protoporphyria (EPP) is the third the majority of porphyria common. It generally occurs in the white but can also occur in the people of any origin. The EP occurs in males and females with roughly equal frequencies. The EP occurs typically in the infants or the infants.

Symptoms beginning usually in childhood. The exposure in the light of the sun causes tinkling, itching or to burn, which can be associated redness and swelling. These symptoms usually occur in a few minutes of exposure of skin in the light of the sun, and often they take hours or days with the resolution. The gallstones cause the characteristic abdominal pain. The damage of liver can carry out to the failure growing of liver, with the ictère and the enlarging of spleen. Some develop the thickening of the surplus of skin their rub them with the fist, and of small scars on the skin exposed to the sun of this type on the cheeks, the nose, and the backs of the hands. There is no treatment for this disorder.

The majority of the treatments must give to your skin additional protection against the sunlight. Protective clothing is also very useful this disorder. The goals of pharmacotherapy are to reduce morbidity and to prevent the beta-carotene of complications. Beta-carotene oral examination reduces photosensitivity in some. Other oral measurements which were employed include the activated carbon and the cholestyramine to stop and prevent the reabsorption of porphyrins. People which develop the gallstones which contain protoporphyrin can have to make them remove surgically.

Topical lubrication of the eyes improves the dry symptoms of eye and can stabilize the visual function. The ultraviolet therapy can be useful. This comprises the exposure three times per week during five weeks to make sure that thickening and the pigmentation resulting from the skin last the remainder of the summer. Severely do not shorten the catch of carbohydrate; a beneficial effect of glucose can modulate the abnormal synthesis of heme. Use of ethanol limit; the alcohol excess was implied in the mortal protoporphyria related to the failure of liver.