Pemphigus is a group of rare autoimmune diseases that cause blistering of the skin and mucous membranes (mouth, nose, throat, eyes, and genitals). Some forms of the disease, including the most common form, may be fatal if left untreated.
Normally, our immune system produces antibodies that attack viruses and harmful bacteria to keep us healthy. In people with pemphigus, however, the immune system mistakenly attacks the cells in the epidermis, or top layer of the skin, and the mucous membranes. The immune system produces antibodies against proteins in the skin known as desmogleins. These proteins form the glue that keeps skin cells attached and the skin intact. When desmogleins are attacked, skin cells separate from each other and fluid can collect between the layers of skin, forming blisters that do not heal. In some cases, these blisters can cover a large area of skin.
It is unclear what triggers the disease, although it appears that some people have a genetic susceptibility. Environmental agents may trigger the development of pemphigus in people who are likely to be affected by the disease because of their genes. In rare cases, it may be triggered by certain medications. In those cases, the disease usually goes away when the medication is stopped.
Pemphigus is not contagious. It does not spread from person to person.
Though there can be a genetic predisposition to develop pemphigus, there is no indication that the disease is hereditary.
Pemphigus affects people across racial and ethnic lines. Research has shown that certain ethnic groups (such as the eastern European Jewish community and people of Mediterranean descent) are more susceptible to pemphigus. A particular type of pemphigus occurs more frequently in people who live in the rain forests of Brazil.
Men and women are equally affected. Research studies suggest a genetic predisposition to the disease. Although the onset usually occurs in middle-aged and older adults, all forms of the disease may occur in young adults and children.
There are several types of pemphigus and other similar blistering disorders. The type of disease depends on where (what layer) in the skin the blisters form and where they are located on the body. Blisters always occur on or near the surface of the skin, which is called the epidermis. People with pemphigus vulgaris, for example, have blisters that occur within the lower layer of the epidermis, while people with pemphigus foliaceus have blisters that form in the topmost layer. The type of antibody that is attacking the skin cells may also define the type of disease present.
A diagnosis of pemphigus has several parts:
Pemphigus is a serious disease, and it is important to do all of these tests to confirm a diagnosis. No single test is right all of the time.
Because it is rare, pemphigus is often the last disease considered during diagnosis. Early diagnosis may permit successful treatment with only low levels of medication, so consult a doctor if you have persistent blisters on the skin or in the mouth. In the most common form of pemphigus (pemphigus vulgaris), the mouth is often the first place that blisters or sores appear.
Pemphigus is a rare disease of the skin; therefore, dermatologists are the doctors best equipped to diagnose and treat people with pemphigus. If you have blisters in the mouth, a dentist can provide guidance for maintaining good oral health. This is important for preventing gum disease and tooth loss.
Treatment for pemphigus vulgaris may involve using one or more drugs. The main goal is to suppress the immune system so that it will stop attacking the tissues. Your doctor may prescribe corticosteroids and immunosuppressive drugs.
For more information on Pemphigus vulgaris