PV is rare - about 2 in 100,000 people develop it each year. Most affected people are middle aged, usually over 50. It is very rare in children. Men and women are equally affected. 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(s) 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, medicines, sunlight, or other environmental factors. There is also an inherited factor which makes some people more prone to 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.

What are the symptoms of pemphigus vulgaris?

Skin blisters. Many blisters may develop on any part of the body. 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.

How does pemphigus vulgaris progress?

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.

The diagnosis may not be clear at first. There are other causes of mouth erosions and skin blisters. Therefore a small sample (biopsy) of skin may be taken. This can be looked at under the microscope and tested to confirm that the blisters are due to PV.

What is the treatment of pemphigus vulgaris?

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

Steroid tablets are the usual treatment. Steroids reduce inflammation and suppress the immune system. A high dose is usually needed 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 treatment from time to time when the blister activity is low (in remission). It 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. (A separate leaflet discusses steroid tablets in more detail.)
An immunosuppressant such as azothiaprine, cyclosporin or cyclophosphamide is also commonly advised. These work by suppressing the immune system. One is usually advised in addition to steroid medication. The two together tend to work better than either alone. Also, the dose of steroid needed may be less. This means that any side-effects from steroids may be less severe. An immunosuppressant is sometimes used alone instead of steroids.
Steroid cream is sometimes used in addition to other treatments. This may keep the dose of steroid tablets lower than would otherwise be needed.
Antiseptic creams may be advised if there are large areas of raw skin. This is to reduce the chance of infection.

Different people may respond best to different combinations of medicines. Other medicines such as gold and dapsone have also been tried with some success. These may be tried in combination with steroid tablets if the above medicines do not work very well.

What is the outlook (prognosis)?

The outlook is variable. PV is a chronic (persistent) condition. With treatment, PV is usually controllable. Less than 1 in 10 affected people die as a result of PV. The main risk of serious illness and death is due to severe infection which may develop on areas of raw skin.

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.

Further help and advice

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

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