Pemphigus is a group of rare autoimmune disorders. It causes large skin blisters. The immune system normally protects against germs and other foreign invaders. In this case, the immune system mistakenly attacks your own skin and mucus membranes. There are three forms of the disease:
The immune system produces antibodies. These cause the skin and mucus membranes to break out in blisters and burn-like sores. What causes the body to attack itself is not known. Sometimes a drug can cause symptoms.
Factors that increase your chance for pemphigus include:
Itching and pain are common symptoms. Pemphigus may occur over a small or large section of the skin. Symptoms differ among the types.
The doctor will ask about your symptoms and medical history. A physical exam will be done. This will include a thorough skin exam. Special care is given to examining the lesions.
Tests may include:
There is no cure. Treatment aims to control the disease and prevent infection of the blistering lesions. The wounds are treated similarly to severe burns . Antibiotics and bandages with petroleum jelly will be used.
Early treatment produces better results. Even with treatment, a series of remissions and relapses usually occurs.
If left untreated, pemphigus can lead to death.
If a drug triggered the disease, the doctor will stop that medication. In some cases, stopping the drug is all that is needed for recovery.
Drugs can usually help control symptoms. The medications can produce serious side effects. Your doctor may order periodic blood and urine tests to check for adverse effects.
Drugs that may be given include the following:
For this procedure, whole blood is removed and the plasma is separated. The packed cells are then suspended in saline are reinjected. This is used in certain cases in combination with immunosuppressive drugs.
Good nutrition helps the body heal and fight disease. Some foods may also make your symptoms worse or trigger the onset of more sores. Some patients notice they are sensitive to garlic, onions, and leeks. Keep track of the foods that seem to cause a reaction in your skin and avoid these foods. Read the labels of all foods to make sure they do not contain small amounts of foods that can set off a reaction.
Soft diets may be necessary if you have oral lesions.
In some cases of paraneoplastic pemphigus, surgical removal of the tumor may improve the disorder or decrease symptoms.
RESOURCES:
American Autoimmune Related Diseases Association, Inc.
http://www.aarda.org/
International Pemphigus Foundation
http://www.pemphigus.org/
CANADIAN RESOURCE:
Canadian Dermatology Association
http://www.dermatology.ca/
Health Canada
http://www.hc-sc.gc.ca/
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Last reviewed January 2009 by Ross Zeltser, MD
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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