Pemphigus Frequently Asked Questions

A. Pemphigus is a group of rare autoimmune diseases that cause blistering of the skin and mucous membranes such as in mouth, nose, throat, eyes, and genitals.

A. There are several types of pemphigus. The type of disease depends on what layer in the skin the blisters form and where they are located on the body. The two main types are:

• Pemphigus vulgaris: It is the most common subtype of pemphigus in the United States and worldwide. It is characterised by blisters and erosions on the skin and mucous membranes, most commonly inside the mouth.
• Pemphigus foliaceus: It is characterised by blistering lesions which usually begin on face and scalp and later on the chest and other parts of the body. The sores are superficial and often itchy, and usually aren't painful.

A. Pemphigus is an autoimmune blistering disease, which means that an individual's immune system starts attacking its cells in the epidermis, or top layer of the skin, and the mucous membranes.

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 disappears when the medication is stopped.

The primary aim of treatment is to decrease blister formation, prevent infections and promote healing of blisters and erosions. Treatment for pemphigus involves using one or more drugs. High doses of oral corticosteroids are the mainstay of medical treatment for controlling the disease. Immunosuppressive drugs are often added to patient's treatment to minimise steroid use. Severe cases of pemphigus may need plasmapheresis or intravenous immunoglobulin (IVIG) treatment to reduce the amount of antibodies in the blood.