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Pemphigus vulgaris is a rare autoimmune disease that causes blistering of the skin and mucous membranes of the mouth, throat and genital area.
The blisters may vary in size, but are thin-walled and fragile. They break easily and leave areas of raw skin that are painful and susceptible to infection. The blisters usually develop in the mouth at first. In some patients, other areas of the body may be affected at a later stage. Sometimes, large areas of skin peel off at just the rub of a finger. The condition may develop at any age, although the usual age of disease onset is in adults between 50 and 60 years.
This condition is diagnosed by the characteristic clinical appearance, with a skin biopsy from the affected area of the mucous membrane of the mouth. Antibody testing using direct and indirect immunofluorescence is also a useful tool to confirm the diagnosis.
There is no cure for pemphigus vulgaris, but symptoms can be kept under control with the use of corticosteroids or immunosuppressants. General treatment may include:
In most cases, pemphigus vulgaris is initially treated with corticosteroids for a few weeks or months to suppress the autoimmune reaction. This helps to heal existing blisters and to prevent the formation of new ones. It usually takes approximately three weeks for new blisters to stop forming, while about eight weeks are required for existing blisters to heal. The treatment is usually taken in tablet form, although injections and creams may also be used. To lower the risk of side effects, the corticosteroid dose is gradually reduced, meanwhile adding another immunosuppressant to replace its action. It may take some time for the best dose to be established. A balance needs to be found between the control of symptoms and the limitation of adverse side effects. It may be possible to eventually stop treatment in a few patients. Most individuals, however, require treatment on an ongoing basis, in order to prevent flare-ups of the condition.
Steroids may cause several unpleasant and even dangerous side effects when used over the long term. These include the following:
As the dose is gradually reduced, it should be possible to limit these side effects, although osteoporosis can be an ongoing problem.
Once symptoms are under control, another immunosuppressant may be used in combination with the low-dose corticosteroid. Examples include cyclosporine, cyclophosphamide, and azathioprine. These medications may also increase the risk of contracting infection, which is why certain precautions should be taken. These include avoiding close contact with people who have an infection or have been exposed to one, avoiding crowded places and informing your doctor of any symptoms that could indicate infection arise, such as fever.
In cases where a steroid and immunosuppressant fail to bring symptoms under control, another medication may be added to the treatment regimen. However, these treatments are not widely available. Some of these medications are described below:
It may be very distressing for people to find out that they have a rare and serious disease. This can trigger feelings of distress, bewilderment, loneliness and fear. A good way to start coping with it is to find out as much information as possible about the condition. To help with this, there are many support groups, which provide a good starting point. They also offer support to people with the condition to help them manage the stress and the pain of the unpredictable remissions and exacerbations.
Other recommendations that may be helpful to manage the condition include the following: