Pemphigus vulgaris is a rare autoimmune disease that causes painful blistering on the skin and mucous membranes. If you have an autoimmune disease, your immune system mistakenly attacks your healthy tissues. Pemphigus vulgaris is the most common type of a group of autoimmune disorders called pemphigus. Each type of pemphigus is characterized by the location where the blisters form.
Pemphigus vulgaris affects the mucous membranes, which are found in areas including the:
This disease usually starts with blisters in the mouth and then on the skin. The blisters sometimes affect the membranes of the genitals. Pemphigus vulgaris can be dangerous. Treatment is essential, and typically it involves the use of corticosteroids to suppress the immune system. The condition can cause serious complications it isn’t treated. Some of these complications can be fatal. The death rate from this disease had averaged 75 percent before corticosteroids were introduced in the 1950s. This has improved dramatically with today’s treatments. A dermatologist will conduct a physical examination of your skin blisters. They will look for an indicator of the condition called Nikolsky’s sign. A positive Nikolsky’s sign is when your skin shears off easily when the surface is wiped sideways with a cotton swab or a finger. Your doctor may then take a biopsy of the blister, which involves removing a piece of tissue for analysis, and look at it under a microscope to confirm the diagnosis. The biopsy may be treated in the laboratory with chemical substances that help your doctor find the abnormal antibodies. Your doctor can use this information to determine the type of pemphigus.