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Pemphigus vulgaris

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Pemphigus vulgaris Content Supplied by NHS Choices

Pemphigus vulgaris (PV) is a rare and serious 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 tends to 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 they 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.

When to seek medical advice

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

It's unlikely that you 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 can 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.

What causes pemphigus vulgaris

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

Treatments for pemphigus vulgaris

The symptoms of pemphigus vulgaris can often be controlled with a combination of medicines that help stop the immune system attacking the body.

Most people will start off 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 from steroid medication, the dose is then gradually reduced and another medication that reduces the activity of the immune system is taken alongside it.

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.

Read more about treating pemphigus vulgaris.

Risk of infected blisters

There is 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, as an infected blister could potentially lead to a very serious infection if left untreated. Contact your GP or dermatologist for advice straight away.

Treating pemphigus vulgaris

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

Most people will need to take steroid medication (corticosteroids) in addition to another immunosuppressant medication. These help stop the immune system damaging healthy tissue.

The main aims of treatment are to:

  • heal the blisters and prevent new ones forming
  • reduce the dose of your medication gradually to the lowest possible dose that still controls your symptoms

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

You may eventually be able to come off your 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 two to three weeks to stop new blisters forming and up to eight weeks for existing ones to heal.

Once your symptoms are under control, your steroid dose will be gradually reduced to the lowest possible dose that can still control your symptoms. This will help reduce the risk of 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 will 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 alone 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. For example, rituximab is relatively expensive and some clinical commissioning groups (CCGs) may not fund it.

'There's definitely life after pemphigus'

Penelope Sherwood was diagnosed with pemphigus vulgaris at the age of 44. She says she couldn't have coped without the support of her family and the Pemphigus Vulgaris Network charity. Penelope now leads a full and rewarding life teaching Japanese embroidery.

"It was August 1992 and I was looking after a toddler. While giving her some peanut butter sandwiches, I had a tiny mouthful.

"As soon as I swallowed it I realised that I had a huge blood blister on my palate. I panicked. Being a dentist, I knew there could be a serious reason for this.

"I was 44 at the time. It took some months to find out what was wrong. During this time my skin itched terribly, my hair fell out and I was extraordinarily tired.

"After various hospital visits and three biopsies, the doctors told me I had pemphigus vulgaris (PV). I have mucosal PV, which means that my immune system attacks all the mucosa: my nose, mouth, oesophagus (food pipe) and vulva.

"My mouth was in a terrible state. I knew how important it was to keep my mouth clean, but this was very painful so I used mouthwashes. My husband was enormously supportive.

"Initially, I was put on an antibacterial medicine called dapsone. It made me lose all energy and it didn't work, although I know it's effective for some people.

"At that point, I knew I was going to have to take steroids. I was sent to the Royal National Orthopaedic Hospital in Middlesex to investigate my bone density, which can be affected by long-term steroid use.

"I was put on intravenous etidronate once a week initially, then once a month. After three to five months I was given Didronel, a new treatment for weak bones. In the meantime, I was put on prednisolone (a corticosteroid) and azathioprine (to suppress my immune system).

"While I was on large doses of prednisolone, I lost a lot of muscle tone. That's improved now, although my eyesight has still not improved.

"I've been off the steroids for five years now. I'm just taking small doses of azathioprine. Every time I stop my medication, I get blisters in my mouth again.

"When I got the blisters, my diet was very bland. I couldn't eat anything sharp or crispy. The blandness and softness of the food was depressing. I ate the occasional crisp or chip, but it would touch the side of my mouth and I'd get a blister.

"Now, I rarely get blisters. I still get the occasional one if I eat raw banana or spicy foods. If I have stewed fruit, I have to balance it with cream to counter the acidity. But I can eat a lot more things than before.

"I still get tired, so I only do one thing outside the flat each day. I have a full life, as long as I limit myself.

"Before I developed pemphigus I painted, but my muscle weakness meant that I couldn't hold a paintbrush any more. A friend suggested I start painting with a needle and thread.

"I now teach Japanese embroidery. It has opened up a new life for me, which is so satisfying. In a way, I'm thankful for the pemphigus because without it I'd never have discovered the embroidery.

"I'd say this to other people with the disease: however difficult it is to control it, there's definitely life after pemphigus."