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PEMPHIGUS

What is Pemphigus ?

Pemphigus is a blistering skin condition which occurs in people between the ages of 20 and 50. It starts as an itchy red rash, and the skin becomes slightly swollen in well-defined areas. Following this, blisters develop in the skin, which are of variable size. These blisters readily burst to leave erosions on the skin. The erosions tend to increase in size with time. In one variety of pemphigus, blisters may be absent and are replaced by surface skin loss. The eruption can occur on any part of the body, and the insides of the mouth are frequently affected.

How does Pemphigus occur ?

Pemphigus is regarded as an auto-immune disease. That is, the body's defence or immune system regards part of the skin as being foreign and mounts a destructive response against it. The part of the skin to which the response is mounted is the "cement" that sticks the cells of the skin together. The inflammation initially creates itching of the skin. It then causes the cells of the skin to separate from each other, producing the blistering.

Why does Pemphigus occur ?

Nobody knows why pemphigus occurs. It may be associated with other auto-immune diseases affecting other organs of the body. Certain drugs which are used for arthritis or tuberculosis may induce a rash very similar to pemphigus.

Treatment Involved for Pemphigus

It is important that the correct diagnosis is established. This is done by taking a small piece of skin under local anaesthetic and examining it under the microscope. Using special tests, the "immune response" against the skin can be identified, which proves the diagnosis. Treatment of pemphigus relies on the use of steroid (cortisone-like) drugs. Steroid tablets are usually started at quite a high dosage. As the skin eruption is brought under control, the dosage is gradually reduced. In some people, the pemphigus flares up once the steroid tablets have been reduced below a certain level. If this occurs, another medicine (called azathioprine) can be used. This works also by suppressing the body's abnormal immune response. Use of this drug allows the steroid tablets to be reduced further. You may require low doses of steroid tablets for months or even years to prevent recurrences of the disease.

During Treatment for Pemphigus

Response to steroid tablets is rapid in pemphigus. The blisters and erosions quickly heal and irritation is relieved. Steroid tablets do have side-effects and can cause a variety of noticeable changes in the body. A common side-effect is fluid retention, with an increase in weight. Very often the face becomes rounded. If steroid tablets are used for long periods of time, they may cause thinning of the bones, resulting in pain in the spine. Diabetes may also develop. Some patients get peptic ulcers or high blood pressure as a result of the steroids. If you notice any new symptoms while on steroids, you should let your doctor know straight away. Steroid tablets can also cause problems within the skin itself, producing a rash like acne on the face, chest, and back, together with stretch marks. For these reasons, the dose of steroid tablets is kept at the lowest possible level. Drugs such as azathioprine are used to enable the doctor to reduce the steroid tablets to the lowest possible dose. However, this type of drug can affect bone marrow, stopping the formation of red and white blood cells. This can produce anaemia and a proneness to infections. Regular blood tests are therefore needed for patients taking this drug.

After Treatment for Pemphigus

Eventually the disease resolves and the tablets can be withdrawn. After this it is unlikely that the pemphigus will recur, but this is always a possibility.

If Pemphigus is Left Untreated

Pemphigus tends to be a progressive disease. If not adequately treated, the rash would become widespread. Because the skin breaks down into erosions, fluid and protein would be lost from the body, and infection could get into the body through the broken skin. This can be extremely dangerous. Before the discovery of steroids and effective treatment of pemphigus, there was a high mortality associated with pemphigus.

Effects on Family of Pemphigus

Pemphigus is a chronic disabling condition and although effective treatment is available, the treatment does carry side-effects which can profoundly affect the individual. Considerable support is needed by family and friends to be able to help the patient cope with the disease.