Pemphigus Vulgaris

What is pemphigus vulgaris?

Pemphigus vulgaris is a skin condition that causes blisters. PV is a serious disease that needs treatment. If not treated, it can be fatal.

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. Pemphigus vulgaris 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: people of Mediterranean, Jewish or Indian origin.

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 a part of the body. In people with PV, antibodies are made against a protein that binds the skin cells together. This antibody attack 'dissolves' the binding between some skin cells and 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 may be an inherited factor which makes some people more prone to develop auto-immune diseases.

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 are common. The blisters burst soon after forming to leave raw painful mouth 'erosions' (like severe mouth ulcers). Some people with PV only get mouth blisters, without any skin blisters. Other people may get mouth blisters as the first symptom of PV.
• Other inner skin areas (mucous membranes) such as the throat, anus, genitals, vagina and front of eye (conjunctiva) are sometimes affected in a similar way to the mouth. If blisters occur in the larynx (voice box), you may get hoarseness. Sometimes blisters can occur in the oesophagus (gullet), which can make swallowing painful.

How does pemphigus vulgaris progress?

Often, it is the mouth blisters which develop first. These soon burst and form painful mouth erosions. The skin blisters may follow some weeks or months later.

If PV is left untreated, the blisters tend to persist and the number of blisters tends to increase. It can become distressing if there are many blisters, as they can burst to leave 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.

If PV is severe you may need treatment in hospital until it improves.

How is pemphigus vulgaris diagnosed?

The diagnosis may not be clear at first. There are other causes of mouth ulcers and skin blisters. Therefore, if your doctor suspects that you have PV, s/he 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.
• A blood test for antibodies. This uses methods called immunofluorescence or ELISA to measure 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 (the antibody 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. The aim is to stop the 'active' (flare up) phase and produce a 'remission' (quiet phase).

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 to start with.)

Steroid treatment can work fairly quickly: symptoms may start to improve within a few days; new blisters may stop in 2-3 weeks; and old blisters may heal in 6-8 weeks.

The dose of steroid is reduced once new blisters have stopped forming. A lower daily dose is usually then needed to stop the blisters coming back. 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 restarted if symptoms flare up again.

Are there side-effects of steroids?
For some people, 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. See separate leaflet called Steroid Tablets which explains their possible side-effects in more detail. Some important points if you are taking long-term steroids, are:

• Long-term steroid treatment should not be stopped suddenly.
• You are more prone to certain infections.
• One possible side-effect is osteoporosis (thinning of bones), so you may be advised to take vitamin D or other medication to help prevent osteoporosis.

Immunosuppressants

An immunosuppressant medication may be advised. These medicines work by suppressing the immune system. They can be used 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. Sometimes an immunosuppressant is used alone instead of steroids. The aim is to find the lowest dose of treatment (or combination of treatments) which control the blisters.

Examples of immunosuppressant medications which may be used for PV are:
cyclophosphamide, azathioprine, ciclosporin, methotrexate or mycophenolate mofetil. Immunosuppressants usually take longer to work than steroids (about 4-6 weeks).

Skin and mouth treatments (topical treatments)

• 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 may be used to help treat mouth blisters and erosions.
• Mouthwashes containing antiseptic or local anaesthetic can also help.
• Wound care treatments such as dressings help raw areas to heal.
• Compresses or soaks using saline (sterile salt water) or certain antiseptics, may be advised if there are large areas of raw skin. This is to reduce the chance of infection and to stop raw skin from becoming too dry.
• If your PV is going through a flare up, you may be advised to protect the skin and mouth from roughness as much as possible. For example, avoid contact sports, eat soft bland food, and use cream or ointment to protect skin from friction.
• Antifungal medication helps if thrush (Candida infection) has got into the mouth, throat or gullet area.

Other treatments

Various other medicines and treatments have been used in recent years with some success. They include: tetracycline antibiotics, dapsone, gold injections, plasmapheresis, extracorporeal photopheresis, intravenous immunoglobulin, 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 or the best combination of treatments to treat PV.

What is the outlook (prognosis)?

If left untreated

Before treatment with steroids became available, up to 8 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 untreated PV is a bit like having widespread skin burns, with the consequent risk of infection and dehydration.)

With treatment

With treatment, blisters usually heal and stop forming, and the risk of death is much reduced. Many patients with PV are able to have long periods of remission (inactive disease). However, treatment does not always work well and side-effects from treatment can sometimes be serious. These days, about 1 in 10 people with PV die of their condition or as a consequence of severe side-effects from treatment.

Further help and advice

Pemphigus Vulgaris Network

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

International Pemphigus & Pemphigoid Foundation

Provides information and support to people living with pemphigus and pemphigoid.
Web: www.pemphigus.org