Pemphigus Vulgaris

What is pemphigus vulgaris?

Pemphigus vulgaris is a skin condition that causes blisters. PV is a serious disease that is often fatal if not treated.

Note: several skin conditions cause blisters and it is important to know exactly which disease you have. There are other types of pemphigus called: pemphigus foliaceous, pemphigus erythematosus, paraneoplastic pemphigus, and benign familial pemphigus. In addition, conditions with similar sounding names also cause blisters. For example, pemphigoid. These different blistering skin conditions vary greatly in their seriousness, outlook and treatment. PV is the most common form of pemphigus and one of the most serious blistering skin conditions.

This leaflet is only about pemphigus vulgaris (shortened to PV from now on).

Who gets pemphigus vulgaris (PV)?

PV is rare - between one and five people in a million develop PV each year in the UK. Most cases develop in people aged over 50. It is very rare in children. Men and women are equally affected. It is more common in certain racial groups. For example, it is more common in people who come from some countries next to the Mediterranean than in people who come from northern Europe.

PV is not infectious and you cannot 'catch' it from an affected person.

What causes pemphigus vulgaris?

PV is an auto-immune disease. The immune system normally makes antibodies to attack bacteria, viruses. and other 'germs'. In people with auto-immune diseases, the immune system also makes antibodies against part or parts of the body. In people with PV, antibodies are made against a protein that binds the skin cells together. Chemical reactions and inflammation in the skin due to this antibody attack 'dissolves' the binding between some skin cells. This causes the cells to separate from each other. Fluid builds up between the separated cells and forms blisters.

It is not known why PV and other auto-immune diseases occur. It is thought that something triggers the immune system to attack the body's own tissues. Possible triggers include viruses, infection, or other environmental factors. There is also an inherited factor which makes some people more prone to develop auto-immune diseases.

People with PV have a slightly higher than average chance of also having other auto-immune diseases such as pernicious anaemia, rheumatoid arthritis, and vitiligo.

(Note: some auto-immune diseases are more serious than others. It depends on what effect the disease can have on the body. For example, PV is a serious life-threatening disease that can be difficult to treat. On the other hand, some other auto-immune diseases are less serious and some are easy to treat.)

What are the symptoms of pemphigus vulgaris?

• Skin blisters. Many blisters may develop on any part of the skin. The most common areas affected are the face, scalp, armpits, groins, and pressure points (buttocks, etc). The blisters can be several centimeters across and are not normally itchy. The blisters are flaccid (loose) and fragile so they easily burst. This leaves raw, red areas of skin which can be very sore and painful. The raw areas of skin heal without scarring, but the affected skin may become more pigmented (darker).
• Mouth blisters also commonly occur. These burst soon after forming to leave raw painful mouth erosions (like severe mouth ulcers). Rarely, PV causes mouth problems alone with no skin blisters.
• Other inner skin areas (mucous membranes) such as the throat, anus, vulva, penis and front of eye (conjunctiva) are sometimes affected in a similar way to the mouth.

In more than half of cases the mouth blisters develop first. These soon burst and form painful mouth erosions. The skin blisters may take several months to appear after the mouth blisters first start. The number of skin blisters usually increases over time.

PV tends to be chronic (persistent). It can become distressing if many blisters develop and burst to leave many areas of raw, painful skin. These raw areas are at risk of infection which can be serious. When PV is active in the mouth or throat it can become difficult to eat or drink. If this occurs you may need nutritional advice or supplements from your GP.

How is pemphigus vulgaris diagnosed?

The diagnosis may not be clear at first. There are other causes of mouth erosions and skin blisters. Therefore, if your doctor suspects that you have PV he or she will refer you to a skin specialist. Tests are usually done to confirm the diagnosis. These include:

• A biopsy (a small sample) of skin may be taken. This is looked at under the microscope and tested to confirm that the blisters are due to PV.
• Indirect immunofluorescence (also called an antibody titre test). This blood test detects and measures the level of the antibody that causes PV (the PV auto-antibody) in your bloodstream. As well as helping to confirm the diagnosis, the level of auto-antibody in your blood gives a good indication of how active the disease is. It may also be measured from time to time to monitor how well treatment is working (as the level goes down when the disease is less active).

What is the treatment of pemphigus vulgaris?

Treatment aims to stop, or greatly reduce, the number of blisters that form.

Steroids
The usual treatment is to take steroid tablets such as prednisolone. Steroids reduce inflammation and suppress the immune system. A high dose is usually needed at first. (Sometimes this is given by injection at first.) The dose is reduced once new blisters have stopped forming. A lower daily dose is usually then needed to prevent blisters forming. The aim is to find the lowest dose needed to control symptoms. The dose needed varies from person to person.

Some people are able to stop steroid tablets from time to time when the blister activity is low (in remission). The tablets can be started again if symptoms flare up again. In some cases, the dose of steroid needed to control the disease is quite high and may cause side-effects. Side-effects from steroids can sometimes be serious, especially if you take high dose steroids for a long time. For example, you are more prone to certain infections if you take regular high dose steroids. (A separate leaflet discusses steroid tablets and their possible side-effects in more detail.)

Immunosuppresants
An immunosuppressant such as azothiaprine, ciclosporin, etc, is also commonly advised. These medicines work by suppressing the immune system. One may be advised in addition to steroid tablets. The two together tend to work better than either alone. Also, the dose of steroid needed may be less if you take an immunosuppressant. This means that any side-effects from steroids may be less severe. An immunosuppressant is sometimes used alone instead of steroids.

Topical steroids
A steroid cream is sometimes used on the skin blisters in addition to other treatments. This may keep the dose of steroid tablets lower than would otherwise be needed. Steroid mouthwashes or sprays are sometimes used to help treat mouth blisters and erosions.

Antiseptic creams
May be advised if there are large areas of raw skin. This is to reduce the chance of infection.

Other treatments
Various other medicines and treatments have been used in recent years with some success. They include dapsone, gold injections, plasmapheresis, intravenous immunoglobulin (IVIg), and rituximab (a monoclonal antibody). These treatments tend to be used when the more usual treatments described above have not worked so well. Sometimes one may also be tried in combination with steroid tablets.

Research continues to find better treatments, and the best combination of treatments to treat PV.

What is the outlook (prognosis)?

If left untreated
Before treatment with steroids became available, up to 9 in 10 people with PV died within a year or so of the disease starting. The cause of death was often serious infection combined with dehydration which easily occurs if you have widespread raw areas of skin. (Having PV is a bit similar to having widespread skin burns with the consequent risk of infection and dehydration.)

With treatment
With treatment, blisters often go and stop forming, and the risk of death is much reduced. However, treatment does not always work well and side-effects from treatment can sometimes be serious. These days, just under 1 in 10 people with PV die of their condition or as a consequence of severe side-effects from treatment.

In summary

• Pemphigus vulgaris is a serious blistering disease.
• Treatments with steroids and other immune suppressants can usually control the disease.
• Treatment aims to balance good control of the disease with the risks and side-effects of medication. The lowest dose that keeps the blisters away is aimed for.
• You may find the disease difficult to cope with, even when symptoms are controlled, as the treatments may cause side-effects.

Pemphigus Vulgaris Network
Flat C, 26 St Germans Road, London SE23 1RJ
Web: www.pemphigus.org.uk

© EMIS and PIP 2005   Updated: December 2005