British Association of Dermatologists
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Introduction
Glossary
What is pemphigus vulgaris?
What types of pemphigus are there?
What areas of the body are affected by pemphigus vulgaris?
What does pemphigus vulgaris look like?
Who tends to have pemphigus vulgaris?
What causes pemphigus vulgaris?
Is pemphigus vulgaris hereditary?
Is pemphigus vulgaris catching?
Is pemphigus vulgaris serious?
Are there any complications with pemphigus vulgaris?
Does anything make pemphigus vulgaris worse?
Will pemphigus vulgaris go away?
How will pemphigus vulgaris be diagnosed?
How can pemphigus vulgaris be treated?
Introduction
What does it mean if I am told my pemphigus vulgaris is in 'remission'?
Managing a pemphigus vulgaris 'flare-up'
What can I do to help myself?
How will pemphigus vulgaris affect my daily life?
I am about to see a health care professional; what do I ask him or her?
Other frequently asked questions (FAQ)
What do I do next?
Where can I get more information?
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>> Introduction

Treatment is important because PV is a serious disease (see ‘Is pemphigus vulgaris serious?’ and ‘Are there any complications with pemphigus vulgaris?’). If PV is left untreated, 3 in every 4 people are likely to die of the disease after one year 8. The main benefit of treatment is that this risk is greatly reduced. With modern treatment, the risk of dying from PV is less than 6% 7,8,9.

When treatment is first started, the main aim of the treatment will be to heal the erosions and blisters that you already have and to stop any new ones forming. This is called ‘disease control’ or ‘disease suppression’. Treatment usually starts with Corticosteroids and then a so-called steroid-sparing drug is usually added later on (see Corticosteroids and Steroid-sparing (or adjuvant) drugs).

There are other less-used drug treatments that are also used in certain circumstances (see Other Treatments).

Once disease control is reached, the aim is to slowly reduce the dose of medication to the lowest dose needed to keep the PV under control 7,16. If your dose has been lowered and your disease is under control, sometimes your doctor will say you are remission, but this can be a confusing term (see‘ What does it mean if I am told my pemphigus vulgaris is in ‘remission?’).

Many patients get good control of their symptoms with drug treatment very quickly. But for others, the disease is unstable and more difficult to control. For these patients, it may take many months, or even years, to find the best type of treatment for them and for the symptoms to become stable.

Even after the disease is under control, it is likely that you will have relapses, known as ‘flare-ups’. If you experience a flare-up of the disease, you will usually need to see your doctor again (see ‘Managing a pemphigus vulgaris flare-up’).

Most of the drugs used to treat PV work by suppressing the immune system because PV is an autoimmune disease (see ‘What causes pemphigus vulgaris?’). These drug treatments can help you get control of the disease but they also have side-effects (see ‘Side-effects of treatments’). However, because PV is a serious condition, it would be extremely dangerous (and painful) to refuse treatment. The medicines are usually given orally (by mouth) but are sometimes given intravenously if necessary (into a vein through a drip).

There are complementary therapies available that may help to reduce the unwanted side-effects from the medicines used to control PV (Complementary medicine) and also other things that you can do to help yourself such as eating a healthy diet (see ‘What can I do to help myself?').

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