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Treatment for pemphigus vulgaris involves using one or more drugs. High-dose oral corticosteroids, such as prednisone or prednisolone, are the main treatment for pemphigus. These are anti-inflammatory medicines that suppress the immune system. High doses are often required to bring pemphigus under control. To minimize the side effects patients may experience, once the disease begins to subside the corticosteroid levels are reduced slowly to the lowest level required to prevent new blisters or sores from appearing. Many patients will go into complete remission with treatment, although this may take a number of years. Other patients will need to continue to take small doses of medication to keep the disease under control. Prednisone is usually taken by mouth, but can also be injected into a vein, muscle, or directly into a blister. The route depends on the type and severity of disease. Usually, a corticosteroid cream will be used directly on the blisters.
To keep the levels of corticosteroid use to a minimum, immunosuppressive drugs are often added to a patient's treatment. These are drugs that stop or slow down the immune system's response to what it sees as an attack on the body. They include:
Other drugs that may be used include:
All of these medications can cause serious side effects. You should see your doctor regularly for blood and urine tests. Be sure to report any problems or side effects you experience to the doctor. With prolonged high-dose corticosteroid therapy, common side effects include susceptibility to life-threatening infections, delayed wound healing, osteoporosis, cataracts, glaucoma, type 2 diabetes, loss of muscle mass, peptic ulcers, swelling of the face and upper back, and salt and water retention. To reduce the risk of osteoporosis, bone density measurements are taken, and patients with low bone density are prescribed medications such as alendronate (Fosamax) or risedronate (Actonel). Extra calcium and vitamin D intake, exercise, and stopping smoking are also recommended. For diabetes caused by steroid use, patients must be on a low sugar diet and may need to take antidiabetic medications.
The immunosuppressive drugs that are used to treat pemphigus can also increase the chances of developing an infection and may cause anemia, a decrease in the white blood cells in the blood, inflammation of the liver, nausea, vomiting, or allergic reactions.
People with severe pemphigus that cannot be controlled with corticosteroids may undergo plasmapheresis, a treatment in which the blood containing the damaging antibodies is removed and replaced with blood that is free of antibodies. Such patients can also be treated with IVIg, or intravenous immunoglobulin, which is given daily for 3 to 5 days, every 2 to 4 weeks for 1 to several months. Plasmapheresis and IVIg are both very expensive treatments, since they require large amounts of donated and specially processed blood. Scientists have reported success in treating difficult cases of pemphigus vulgaris with a combination of IVIg and rituximab, a cancer medication.
The treatment prescribed will depend on the type of pemphigus and the severity of the disease. Work closely with your doctor to devise the treatment regimen that works best for you. Because the medications used to treat pemphigus are strong medications with potentially serious side effects, every doctor that you see should be made aware of the type and amount of medications you are taking.
It may take several months to years for the ulcers and blisters of pemphigus vulgaris to disappear after treatment has begun because circulating antibodies remain in the blood for a long time. Lesions in the mouth are particularly slow to heal. Blisters in the mouth can make brushing the teeth painful, leaving you prone to gum disease and tooth loss. A dentist can offer approaches that enable you to maintain healthy teeth and gums. Avoiding spicy, hard, and acidic foods will help, since those foods can irritate or trigger the blisters. If you are taking corticosteroids, you should receive advice for maintaining a diet low in calories, fat, and sodium, and high in potassium and calcium.