Pemphigus vulgaris - Treatment

Treatment-Pemphigus vulgaris




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. For example, rituximab is relatively expensive and some clinical commissioning groups (CCGs) may not fund it.

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, particularly before eating or brushing your 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 immediately if you develop symptoms of an infection, such as a build-up of pus under the skin, or your skin becoming very painful, hot and red
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