Pemphigus vulgaris is a very serious skin disease that causes blisters. It is rare. Pemphigus vulgaris is more serious than most other blistering skin conditions. Most people with pemphigus vulgaris died before treatment became available. These days most cases can be controlled with treatment. Treatment is usually with steroid medicines and other medicines to suppress the immune system. A skin specialist should supervise treatment.
What is pemphigus vulgaris?
Pemphigus vulgaris (PV) is a rare skin condition that causes blisters. PV is a serious disease that needs treatment. If it is not treated, it is often fatal.
Note: several skin conditions can 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.
There are also conditions with similar sounding names that cause blisters. For example, pemphigoid. These different 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 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 groups of people - those from the Mediterranean, Indian subcontinent or of Jewish origin. PV is not infectious and you cannot catch it from an affected person.
What causes pemphigus vulgaris?
PV is an autoimmune disease. The immune system normally makes antibodies to attack bacteria, viruses. and other germs. In people with autoimmune 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. As a result, the binding between many skin cells is broken and 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 autoimmune 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 autoimmune 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 centimetres 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 are sometimes affected in a similar way to the mouth. If blisters occur in the voice box (larynx), you may get hoarseness. Sometimes blisters can occur in the 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, which are like ulcers. 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, 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.
- 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. When the antibody level goes down, 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). There are various types of medicines used in PV. Unfortunately, there is not enough evidence (yet) to say definitely which treatment works best. A specialist will advise on the most up-to-date thinking on the best treatment. Treatment options include the following:
The usual treatment is to take steroid tablets such as prednisolone. Steroids reduce inflammation (redness and soreness) and suppress the immune system. A high dose is usually needed at first and this may be given by injection. 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. 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. They are more likely 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:
- You should not suddenly stop taking steroids if you have been taking them long-term.
- You are more prone to certain infections.
- One possible side-effect is thinning of bones (osteoporosis). You may be advised to take vitamin D or other medication to help prevent osteoporosis.
An immunosuppressant medicines 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 controls the blisters.
Examples of immunosuppressant medicines 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 (usually drops or pastilles) helps if thrush infects the mouth, throat or gullet area.
Various other medicines and treatments have been used with some success. Plasmapheresis, intravenous immunoglobulin and rituximab (a monoclonal antibody) may be tried if your PV does not respond to high doses of steroids. Sometimes they 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. Dehydration can easily occur 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, blisters usually heal and stop forming, and the risk of death is greatly 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. About 1 in 10 people with PV die of their condition or as a consequence of severe side-effects from treatment. Older people are more likely to have side-effects or die as a result of PV.
Further help and advice
Pemphigus Vulgaris Network
Flat C, 26 St German's Road, London SE23 1RJ
Tel: 020 8690 6462 Web: www.pemphigus.org.uk
International Pemphigus & Pemphigoid Foundation
Provides information and support to people living with pemphigus and pemphigoid.
Dr Tim Kenny
Dr Hayley Willacy
Dr Tim Kenny