Introduction 

Pemphigus vulgaris (PV) is a rare and serious condition that causes painful blisters to develop on the skin as well as inside the mouth, nose, throat, anus and genitals.

The blisters are fragile and can easily burst open, leaving painful areas of raw unhealed skin.

In most people with PV, blisters first appear in the mouth and may later affect the skin. Blisters in the mouth turn into sores, making eating, drinking and brushing teeth very painful. If they spread to the larynx (voice box), the voice can become hoarse.

PV will cause delicate blisters to appear all over the skin if the skin is affected. These blisters easily burst to reveal red sores. The sores can crust over and form scabs, which can eventually discolour the skin. Sores may join together to form large areas of raw-looking skin.

The blisters can fade and flare up later, and it is impossible to predict how severe a flare-up might be.

What causes pemphigus vulgaris?

PV is what is known as an autoimmune condition. This means the immune system (the body's defence against infection) malfunctions and starts attacking healthy tissue.

In cases of PV, the immune system attacks cells found in a deep layer of skin as well as the cells found in the mucous membrane (the protective lining of the mouth, nostrils, throat, genitals and anus). This causes blisters to form in the affected tissue.

It is unclear why the immune system goes wrong in this way. PV could be caused by a combination of both genetic and environmental factors. What is known is that PV is not contagious and cannot be passed from one person to another.

Read more about the possible causes of pemphigus vulgaris.

Treating pemphigus vulgaris

There is currently no cure for PV, although the symptoms respond well to high doses of steroid medication (corticosteroids).

Using a high dose of steroids over a long period of time can cause serious side effects. When the symptoms have cleared, low doses are used in combination with other medicine. This is known as steroid-sparing therapy, and it prevents some of the dangerous side effects associated with long-term steroid use.

If long-term doses of corticosteroids are not effective, other treatment options may be considered, including:

  • other medications, such as azathioprine, mycophenolate mofetil or, very rarely, rituximab infusion
  • plasmapheresis
  • intravenous immunoglobulin therapy

Read more about treating pemphigus vulgaris.

Complications

A possible complication of PV is the blisters becoming infected. In some cases, infection can quickly spread into the blood (sepsis) and affect the whole body, which can be life threatening.

Other complications include side effects associated with the long-term use of corticosteroids and immunosuppressants.

Read more about the complications of pemphigus vulgaris.

Who is affected?

PV is a rare condition. It is estimated that around 3 in every 100,000 people are diagnosed with PV.

Most cases first develop in older adults between the ages of 50 and 60, although PV can affect people of all ages, including children. Both sexes are affected equally.

PV is slightly more common in people of Jewish, Indian and Mediterranean descent.

Outlook

The outlook for people with PV has improved significantly since the introduction of steroids, although the complications associated with the condition can be life threatening.

Most people with PV experience flare-ups when symptoms are particularly bad and then periods when they experience no or very few symptoms (this is known as remission). In some people, the symptoms may go away permanently (complete remission).

However, living with a long-term painful condition can be distressing and affect you emotionally.

Aside from medication, there are also steps you can take to help you cope better, such as exercise and relaxation techniques.

The NHS also runs pain management courses designed to help people with long-term painful conditions learn to live with their pain. 

Read more about living with pain.

Skin blisters caused by pemphigus vulgaris 

Other blistering conditions

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

  • pemphigus foliaceus – the 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, where 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.

Long-term conditions

Living with a long-term condition, including healthcare, medicines and support

Page last reviewed: 21/02/2014

Next review due: 21/02/2016