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