Pemphigus vulgaris

Introduction 

Pemphigus vulgaris (PV) is a rare disease that causes severe blistering of the skin and the mucous membranes lining the mouth, nose, throat and genitals. The mouth is almost always affected, which can make eating and brushing of teeth very painful.

PV is an autoimmune disease, which means that the body's immune system attacks its own cells and organs. In PV, the skin cells are attacked (see Causes for more information).

PV is not infectious, so the condition cannot be passed from person to person.

PV is a relapsing disease, which means that symptoms can fade and then flare up later. You can never predict how severe each flare-up will be.

The disease is potentially life-threatening, and there is no cure. However, symptoms can be controlled with intense immunosuppressive treatment (medication that weakens the immune system; see Treatment for more information).

Who is affected?

PV affects men and women of all ages and races. It most commonly starts in adults aged 50-60.

Outlook

PV has no cure, so many people have to stay on low doses of steroid medication for the rest of their lives in order to keep symptoms under control.

However, some people find that their symptoms eventually get better and do not flare up at all when treatment is stopped. This is known as being in complete remission.

Last reviewed: 16/12/2009

Next review due: 16/12/2011

What are these?

Other blistering conditions

There are many different diseases that cause blistering. Some of them are less common types of pemphigus, such as:

  • Pemphigus foliaceus. Blisters and sores are not as painful as in pemphigus vulgaris (PV). They do not occur in the mouth, but usually appear on the face and scalp, and later on the chest and back.
  • Paraneoplastic pemphigus. The most serious and least common type of pemphigus. Blisters sometimes affect the lungs. It usually occurs in people who have been diagnosed with cancer.

PV is one of the most serious blistering diseases.