Overview

Pemphigus vulgaris (PV) is a rare and serious (potentially life-threatening) condition that causes painful blisters to develop on the skin and lining of the mouth, nose, throat and genitals.

The blisters are fragile and can easily burst open, leaving areas of raw unhealed skin that are very painful and can put you at risk of infections.

There's currently no cure for pemphigus vulgaris, but treatment can help keep the symptoms under control.

The condition can affect people of all ages, including children, but most cases develop in older adults between the ages of 50 and 60. It isn't contagious and can't be passed from one person to another.

Symptoms of pemphigus vulgaris

The blisters usually develop in the mouth first, before affecting the skin a few weeks or months later.

There may be times when the blisters are severe (flare-ups), followed by periods when they heal and fade (remission). It's impossible to predict when this might happen and how severe the flare-ups will be.

Blisters in the mouth often turn into painful sores, which can make eating, drinking and brushing teeth very difficult. The voice can become hoarse if the blisters spread to the voice box (larynx).

Sores on the skin can join together to form large areas of painful, raw-looking skin, before crusting over and forming scabs. They don't usually leave any scars, although affected skin can occasionally become permanently discoloured.

As well as getting blisters in the mouth, they can also develop in other areas of the digestive system's soft tissue lining, including the nose, throat, anus, genitals and vagina. The thin membrane that covers the front of the eye and inside of the eyelids (conjunctiva) can also be affected.

When to get medical advice

See your GP if you have severe or persistent blisters or sores in your mouth or on your skin.

It's unlikely you'll have pemphigus vulgaris, but it's a good idea to get your symptoms checked out.

If your GP thinks your symptoms could be caused by a serious condition such as pemphigus vulgaris, they may refer you to a dermatologist (skin specialist) for some tests.

The dermatologist will examine your skin and mouth, and may remove a small sample (biopsy) from the affected area so it can be analysed in a laboratory. This can confirm whether you have pemphigus vulgaris.

A blood test can also be used to check for antibodies (infection-fighting proteins) in your bloodstream, to help confirm the diagnosis.

What causes pemphigus vulgaris?

Pemphigus vulgaris is an autoimmune condition. This means that something goes wrong with the immune system (the body's defence against infection) and it starts attacking healthy tissue.

In pemphigus vulgaris, the immune system attacks cells found in a deep layer of skin, as well as cells found in the mucous membrane (the protective lining of the mouth, nostrils, throat, genitals and anus). This causes blisters to form in the affected tissue.

It's unclear what causes the immune system to go wrong in this way. Certain genes have been linked to an increased risk of pemphigus vulgaris, but it doesn't tend to run in families.

Treatments for pemphigus vulgaris

Pemphigus vulgaris is a long-term (chronic) condition that can't be cured.

However, the symptoms can often be controlled using a combination of medicines that help stop the immune system attacking the body.

Most people start by taking high doses of steroid medication (corticosteroids) for a few weeks or months. This helps stop new blisters forming and allows existing ones to heal.

To reduce the risk of side effects of steroids, the dose is gradually reduced and another medication that reduces the activity of the immune system is taken as well as the steroid.

It may eventually be possible to stop taking medications for pemphigus vulgaris if the symptoms don't come back, although many people need ongoing treatment to prevent flare-ups.

Risk of infected blisters

There's a high risk of blisters caused by pemphigus vulgaris becoming infected, so it's important to look out for signs of infection.

Signs of an infected blister can include:

  • the skin becoming painful and hot
  • yellow or green pus in the blisters
  • red streaks leading away from the blisters

Don't ignore these signs – an infected blister could potentially lead to a very serious infection if left untreated. Contact your GP or dermatologist for advice straight away.

Help and support

Being told you have a rare, serious condition can be a confusing, frightening and sometimes lonely experience.

It can help to find out as much as you can about the condition and how best to cope with it.

A good place to start is the Pemphigus Vulgaris Network, a UK support group for people with pemphigus vulgaris.

NHS support is also available to help you deal with the physical and emotional effects of living with long-term pain.

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Treatment

There's currently no cure for pemphigus vulgaris (PV), but treatment can help keep the symptoms under control.

The main aim of treatment is to heal the blisters and prevent new ones forming.

Steroid medication (corticosteroids) plus another immunosuppressant medication are usually recommended. These help stop the immune system damaging healthy tissue.

You may eventually be able to stop taking medication if your symptoms disappear and don't come back when treatment is stopped. However, many people will need to keep taking a low dose.

Steroid medication

Steroid medication can help reduce the harmful activity of the immune system in a short space of time. It's usually taken as a tablet, although creams and injections are also sometimes used.

You usually start on a high dose to get your symptoms under control. This can lead to a noticeable improvement within a few days, although it usually takes 2 to 3 weeks to stop new blisters forming and 6 to 8 weeks for existing blisters to heal.

Once your symptoms are under control, your steroid medication will gradually be reduced to the lowest possible dose that can still control your symptoms. This will help reduce the risk of side effects.

It can take a while to find the best dose for you. It may take a few months to reach a balance between controlling your symptoms and limiting unpleasant side effects.

Side effects

If taken for a long time at high doses, steroid medication can have a range of unpleasant side effects, such as:

Most of these side effects should improve if you're able to reduce your dose. However, osteoporosis can be a lasting problem.

Read more about the side effects of steroids medication.

Other immunosuppressants

Once your symptoms are under control, other immunosuppressant medications may be taken alongside a low dose of steroids.

Medicines that may be used include azathioprine, mycophenolate mofetil, ciclosporin and cyclophosphamide. These are usually taken as tablets.

Side effects

Like steroids, these medicines can make you more vulnerable to infection, so you'll need to take precautions when taking them, such as:

  • avoiding close contact with someone known to have an active infection, such as chickenpox or flu
  • avoiding crowded places when possible
  • telling your GP or dermatologist immediately if you develop symptoms of an infection, such as a high temperature (fever)

Other possible side effects include:

  • your skin becoming vulnerable to the effects of sunlight
  • birth defects if the medication is taken during pregnancy

Additional treatments

Several other treatments are sometimes used in combination with steroid medication and other immunosuppressants if these medications don't fully control your symptoms.

These include:

  • tetracycline and dapsone antibiotic tablets that can alter the activity of the immune system
  • rituximab –  a new type of medication that helps stop your immune system attacking your skin cells; it's usually given by drip directly into a vein over a few hours
  • plasmapheresis – where your blood is circulated through a machine that removes the antibodies that attack your skin cells
  • intravenous immunoglobulin therapy – where normal antibodies from donated blood that temporarily change how your immune system works are given through a drip

These treatments don't tend to be used very often and aren't always widely available.

Self-help tips

To help cope with pemphigus vulgaris:

  • use a soft toothbrush and avoid spicy, crispy or acidic foods if you have blisters in your mouth
  • take painkillers or use anaesthetic mouthwashes to relieve mouth pain, especially before eating or brushing teeth
  • practice good oral hygiene – brush your teeth regularly and use antiseptic mouthwash
  • avoid activities that could damage your skin, such as contact sports
  • keep cuts or wounds clean to prevent serious skin infections
  • contact your GP or dermatologist if you develop symptoms of an infection, such as pus building under the skin, or the skin becoming very painful, hot and red
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The information on this page has been adapted by NHS Wales from original content supplied by NHS UK NHS website nhs.uk
Last Updated: 17/09/2019 11:00:20