Overview
Pemphigus vulgaris
Pemphigus vulgaris is a rare long-term condition caused by a problem with the immune system. It causes blisters in the mouth and on the skin. It can be very serious, but treatment can help control it.
Check if you have pemphigus vulgaris
Pemphigus vulgaris causes blisters that burst easily and leave very sore patches.
The most common area affected is inside the mouth and throat, which can make swallowing painful.
Many people also get blisters on their skin. These usually appear a few months after the mouth is affected.
The blisters and sore areas can cover a large area of the body. Sometimes they can also affect the eyes, genitals or anus
If you're not sure it's pemphigus vulgaris
Pemphigus vulgaris can have similar symptoms to more common conditions, such as impetigo and hand, foot and mouth disease.
Do not try to diagnose yourself. See a GP if you're worried.
See a GP if:
- you have blisters or sore patches in your mouth or on your skin that do not go away or keep coming back
Treatments for pemphigus vulgaris
If a GP thinks you could have pemphigus vulgaris, they can refer you to a skin specialist (dermatologist) for tests and treatment.
Blisters caused by pemphigus vulgaris usually come and go over time. There's no cure, but treatment can help.
The main treatments are:
- steroid tablets
- other medicines such as azathioprine, methotrexate and rituximab
It usually takes a few weeks for the blisters to heal. Once this happens, you'll probably need treatment for several years to help stop the blisters coming back.
It may be possible to stop treatment eventually. If the blisters come back, you may need to repeat treatment.
Things you can do if you have pemphigus vulgaris
If you have pemphigus vulgaris, there are some things you can do to help ease the symptoms and reduce the risk of further problems.
Do
- clean your teeth using a soft toothbrush and mint-free toothpaste
- use antiseptic or painkilling (anaesthetic) mouthwash (try to avoid mouthwashes that contain alcohol)
- keep any sore patches clean to reduce the risk of infection
- have regular dental check-ups
Don’t
- do not eat spicy, acidic or hard foods if you have blisters in your mouth
- do not do activities that could damage your skin (such as contact sports) if you have blisters on your skin
Complications of pemphigus vulgaris
Pemphigus vulgaris can lead to other problems, such as:
- blisters and sore patches becoming infected, which can cause life-threatening problems such as sepsis
- weight loss and malnutrition because of pain when eating and drinking
- tooth decay and gum disease because it can be painful to look after your teeth and gums
- pain during sex and pain when peeing or pooing (if your genitals or anus are affected)
You'll have regular check-ups to look for these problems and may be referred to other specialists (such as a dietitian) if needed.
Ask for an urgent GP appointment or get help from NHS 111 if:
- your blisters or sore patches are very painful or feel hot
- there is yellow or green pus coming from your blisters or sore patches
These are signs of an infection, which needs to be treated quickly.
You can call 111 or get help from 111 online.
Causes of pemphigus vulgaris
Pemphigus vulgaris is caused by a problem with the immune system, which is the body’s defence against infection.
Instead of attacking germs, something goes wrong with the immune system and it attacks the skin by mistake. This damages the skin and causes blisters to appear.
It’s not clear why this happens. It’s not passed on in families and it’s not caused by an infection, so it cannot spread to other people.
Support if you have pemphigus vulgaris
Living with pemphigus vulgaris can be very difficult.
As well as support from your care team, it may help to get information and support from groups and charities such as:
Pemphigus Vulgaris Network – a UK support group for people with pemphigus vulgaris
PEM Friends – a UK support group for people with pemphigus vulgaris and related conditions
International Pemphigus and Pemphigoid Foundation – a global support group for people with pemphigus vulgaris and related conditions
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; you should also have regular dental check-ups
- 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