This information is provided by the National Institutes of Health (NIH) Genetic and Rare Diseases Information Center (GARD).
Pemphigus vulgaris is an autoimmune disorder that involves blistering and erosion of the skin and mucous membranes. It occurs almost exclusively in middle-aged or older people. The primary lesion of pemphigus vulgaris is a soft blister filled with clear fluid that appears on healthy or irritated skin. Many cases begin with blisters in the mouth, followed by skin blisters that may come and go. The blisters inside the mouth can make it hard for the person to eat. The rupture of blisters on the skin may be painful and limit the person’s daily activities. Complications due to infections can be serious and the damaging nature of the blisters can cause loss of body fluids and protein. The exact cause of pemphigus vulgaris is unknown, but the blisters in pemphigus vulgaris are associated with the binding of antibodies to the skin cells. Treatment is aimed at reducing symptoms and preventing complications, and may include the use of corticosteroids, immunosuppressive drugs, and more recently immunotherapy. Pemphigus vulgaris may require long term treatment to keep it in remission.
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