Pemphigus vulgaris (PV) is a rare and serious condition that causes painful blisters to develop on theskin 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 conditioncan affect people of all ages, including children, but most cases develop in older adults between the ages of50 and 60.It isn't contagious and can't be passed from one person to another.

Symptoms of pemphigus vulgaris

Theblisters 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 severethe flare-upswill 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 canjoin together to form large areas of painful, raw-looking skin, before crusting over and forming scabs.They don't usually leave any scars , althoughaffected skin canoccasionally 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, andmay 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 vulgarisis what's known as an autoimmune condition. This means that something goes wrong withthe 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 causesthe immune system togo wrong in this way. Certain genes have been linked to an increased risk ofpemphigus 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) fora few weeks or months. Thishelpsstopnew 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 ofthe immune system is taken alongside it.

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

Contact your GP or dermatologist for advice straight away.

Content supplied by the NHS Website

Medically Reviewed by a doctor on 28 Nov 2016