Treatment for pemphigus vulgaris (PV) usually involves taking a combination of two types of medication – steroid medication (oral corticosteroids) and immunosuppressants.
Both of these types of medication can prevent your immune system from damaging healthy tissue.
It can take a while to determine the best dose of these medications. There is often a fine balance to successfully controlling symptoms while limiting unpleasant side effects, so it may take some months until this balance can be achieved.
The aim of treatment is:
- To heal the blisters and prevent new ones from forming. It usually takes up to eight weeks for the blisters to heal and two to three weeks to prevent new ones from forming.
- To reduce the medication gradually to the lowest possible dose that will still control symptoms.
Oral corticosteroids (steroid tablets)
Corticosteroids are usually effective in reducing the harmful activity of the immune system in a short space of time.
They contain synthetic versions of hormones that can block some of the chemical reactions of the immune system. In blocking these reactions, further damage to the skin can usually be prevented.
When corticosteroids were first introduced as treatment for PV in the early 1950s, it led to a big fall in the number of deaths from the condition.
First stage: high dose
You usually start on a high dose of prednisolone (60-100mg a day). The dose will depend on how serious your condition is.
If you need more than 100mg a day, pulse therapy is an option. Pulse therapy involves you being given high doses of methylprednisolone plus cyclophosphamide through a drip in your arm.
Pulse therapy sessions are given three days a month for at least six months.
Some people notice an improvement in their symptoms within days of taking corticosteroids. On average, it takes two to three weeks for the blistering to stop.
Next stage: lowering the dose
Once your symptoms have been relieved, your medication is gradually reduced to the lowest possible dose that can still control your symptoms.
The lowest possible dose is used because corticosteroids have a wide range of unpleasant side effects, such as:
- weight gain
- acne
- mood changes, for example you may become aggressive, irritable and short tempered with people
- rapid mood swings, such as feeling very happy one minute and then very sad and weepy the next
Eventually, you may be able to come off corticosteroids altogether if your symptoms have disappeared and do not flare up when treatment is stopped. However, many people will always need to take a low dose of corticosteroids to keep symptoms under control.
Long-term corticosteroid use does increase the risk of you developing a range of health conditions, such as:
Read more about corticosteroids including how they work and their possible side effects.
Immunosuppressants
As their name suggests, immunosuppressants suppress or ‘dampen down’ the immune system.
By combining them with corticosteroids it is often possible to reduce the dosage of your steroid medication, meaning you are less likely to experience side effects and long-term complications such as osteoporosis. This is known as steroid-sparing therapy.
The most commonly used immunosuppressants in the treatment of PV include:
- azathioprine
- cyclophosphamide
- mycophenolate mofetil
These medications are usually taken in tablet form. If one immunosuppressant does not seem to be effective then another one can be tried.
Immunosuppressants 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, especially if it is chickenpox or shingles, as the virus that causes these two infections could make you feel very ill
- if possible avoiding crowded places
- reporting any possible symptom of infection, such as a high temperature or swelling, immediately to your GP or dermatologist
In addition, azathioprine can make your skin more vulnerable to the effects of sunlight so you should wear sunscreen on bright days.
Immunosuppressants are not suitable for pregnant women as they can cause birth defects. If you are a sexually active woman you should use a reliable method of contraception while taking immunosuppressants.
Other treatment
There are several other treatments that are sometimes used in combination with corticosteroids and immunosuppressants.
These include:
- dapsone – a type of medication that can help prevent blisters from becoming infected with bacteria
- colchicine – which is a medication that can reduce levels of inflammation in the body and can help prevent new blisters from forming
- tetracycline – a type of antibiotic that, like dapsone, can be useful in preventing infections
Rituximab
Rituximab is a new type of medication that works by targeting what are known as B cells. These are a type of white blood cell that the immune system uses to attack healthy skin tissue in cases of PV.
Research published in 2011 concluded that rituximab is an effective alternative to conventional treatment in cases where it is ineffective or causes serious side effects.
Rituximab is administered directly into your vein over the course of a few hours.
It is still unclear what the most effective method of prescribing rituximab is. Some doctors have tried using it once a week over the course of four weeks.
Others have suggested it should be used six times over the course of two months followed by four more doses once a month. Further research is needed.
It is common to experience flu-like symptoms when you are being treated with rituximab. Possible symptoms include:
- headache
- fever or chills
- fatigue
- muscle pain
You may be given additional medication to prevent or lessen side effects, although the side effects should improve as your body gets used to rituximab.
Current evidence suggests that around 1 in 14 people will experience more serious side effects after taking rituximab, typically an infection such as pneumonia or sepsis (infection of the blood or 'blood poisoning’).
A practical disadvantage of rituximab is that it is a relatively expensive medication and some primary care trusts may be unwilling to fund treatment.
If you think you could benefit from rituximab you should discuss the issue with your dermatologist.
