sexta-feira, 31 de agosto de 2012

Porphyria cutanea


The cutaneous porphyria is a disease that causes blisters, excess hair, swelling and skin necrosis. Can lead to teeth and nails and red after exposure to sunlight urine turns blue, pink, brown or black. Today it is thought that the disease is linked to many legends of vampires and werewolves where the sufferer was forced to live away from society for being mistaken for a monster.
The disease is part of a group of disorders known as porphyrins which lead to mental and physical problems due to overproduction of certain enzymes in the body. The name of the disease comes from the Greek "porphura" meaning "purple pigment".
Treatment
Some of the medications used to treat a sudden attack (acute) of porphyria may include:
• administered by intravenous hematin
• Analgesics
• Propranolol to control the heartbeat
• Sedatives to make you feel sleepy and less anxious
Other treatments include:
• Supplements of beta-carotene
• Chloroquine
• Fluids and glucose to boost carbohydrate levels, which helps to limit the production of porphyrins
• Withdrawal of blood (phlebotomy)
Depending on the type of porphyria you present, your doctor may advise you to:
• Avoid alcohol
• Avoid drugs that can trigger an attack
• Avoid skin lesions
• Avoid sunlight as much as possible and use sunscreen when you are outdoors
• Follow a diet with large amounts of carbohydrates

Prevention
Consult a geneticist may be helpful for people who want to have children and who have a family history of any type of porphyria.

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