Q1: I’ve been diagnosed with pemphigus vulgaris and started treatment. How long will it be before it works?

This varies from person to person. Once your doctor has found the drug regimen that works best for you, PV often takes up to six months to become stable and for you to be on the lowest effective dose. But PV is difficult to control and it may take longer. Sometimes it can take up to a couple of years for flare-ups to settle down.

Q2: How long will I have to stay on the drugs?

Most people need to stay on a ‘maintenance’ dose permanently to keep their PV under control. Maintenance doses vary from person to person and your doctor will be aiming to get the dose as low as possible whilst keeping the disease controlled. Occasionally people manage to come off all drugs, without any symptoms and this may last for quite a few years, or even permanently.

Q3: I’m still on Prednisolone (10mg a day) and Azathioprine (50mg) and I still get occasional lesions. But my doctor says I’m in remission. If I’m in remission why do I still need to take any drugs and why do I still get lesions?

When your doctor says your disease is in remission, they simply mean that the treatment is keeping the disease ‘under control’. So you still need to take your prednisolone and azathioprine to keep the disease under control. True disease remission where there is absolutely no disease activity (so no drugs needed) is rare.

Your doctor is trying to ensure you are taking the lowest effective dose (to keep the side-effects down) so this might mean you get an occasional lesion.

Q4: The drugs make me feel nauseous. Is there anything that helps?

There are some acupuncture points on the inside of the wrist that seem to help nausea. Try using ‘Travel Bands’ (developed to help travel sickness). They are cheap and can be purchased from large chemists. There are very clear instructions telling you how to use them. You may have to leave them on for several hours to get the best effect.

Some people find ginger herb tea helps minimise the nausea feeling. You can buy ginger and lemon tea bags in most health food shops, or add a slice of fresh ginger to hot water if you prefer. If the nausea is really difficult, see if your GP can prescribe anti-emetics (anti-sickness tablets) to help.

Q5: Will my children get pemphigus vulgaris?

Although genetic factors are probably involved in PV, it’s not hereditary. It is very rare for more than one person in a family to have PV.

Q6: I’m on steroids and/or immunosuppressives and I’m exhausted most of the time. Is there anything I can do?

Unfortunately lots of people do find the drugs (and the illness) make them very tired. There’s nothing you can do to stop this but you'll probably find it easier if you can try and structure daily life to include some resting time.

Make sure the people around you understand the problem.

Q7: I’ve started taking steroids and I’ve gained lots of weight and have a puffy face. What can I do?

The puffed up face does go down as the steroid dose reduces. General weight gain is more difficult and weight control feels like a constant struggle. Nutritionists advise a diet with high protein to low carbohydrate and low fat. See the section ‘How will pemphigus vulgaris affect my daily life?’ for more information and suggestions on reading. Nearly everyone gains weight no matter how disciplined they are about food. It’s important not to get too worried about it, particularly at the start of treatment when the crucial thing is to get the PV under control. For further reading on steroids see ‘Where can I get more information?’.

Q8: I don’t know if it’s safe to go abroad or be in the sun?

If you feel well enough to go on holiday, why not go and enjoy it. But if you’re in the middle of a flare-up it’s probably too risky to go abroad. If your condition flares up while you are abroad, you might need to be hospitalised and there might not be appropriate medical facilities in the place you are visiting. So it would be sensible to visit places which are not too remote and where hygiene and medical standards are good.

Also, if you need travel vaccines, you must check that with your doctor that they are suitable for people on immunosuppressive treatment. There have been a few reports of vaccines aggravating pemphigus.

Don’t forget you will need to declare your PV on your holiday insurance and you may not be able to get insurance on any pemphigus-related situations that come up.

Being in the sun is potentially dangerous. Not only is sunbathing now thought to increase your chances of skin cancer, but ultra-violet light may also be a potential trigger for pemphigus (see ‘Does anything make pemphigus vulgaris worse?’). Also, if you are taking immunosuppressant drugs you need to make sure you don’t sunbathe because your chance of getting skin cancer is increased. So try to stay in the shade, wear a hat and wear clothes that keep you cool and cover your arms and legs. Remember to use a sunscreen of SPF20 or above.

Lots of people with pemphigus vulgaris have gone abroad, taken sensible precautions and been fine.

Q9: I’m having a flare-up but when I phoned my hospital department the receptionist couldn’t give me an appointment. How do I know when I need to see a doctor?

Once the pemphigus is controlled and you’ve been living with it for a while, you’ll probably be able to recognise a bad flare-up, which may very well need your drug dose to be temporarily increased.

Unless you already have specific instructions from your dermatologist on what to do, contact the hospital and arrange to see the doctor as soon as possible. Most hospital Dermatology Out-Patient Departments in the UK have what is called an ‘S.O.S’ arrangement to enable pemphigus vulgaris patients with a bad flare-up to see someone quickly. You may have to remind the receptionist you are an S.O.S. patient. Alternatively try to get an earlier appointment with your dermatology nurse. If you still have no joy, ring the consultant’s secretary and leave a message for the consultant to say that you are having a flare-up.

Q10: Will I get osteoporosis from the steroids I have to take?

If you’re on long-term steroids it’s common practice to be given a calcium and vitamin D supplement to protect against osteoporosis. There are also special bone-building drugs called bisphosphonates that can be taken to help prevent bone loss. These are given to most patients over 65 and to many younger patients depending on individual circumstances. Ask your GP or dermatology consultant to arrange for you to see the appropriate specialist. You may need regular monitoring to make sure all is well. Medical evidence suggests that bone damage can occur within the first three months of steroid treatment ¹⁷.

Q11: I’m taking immunosuppressants. Does this mean I will always get lots of infections?

Once the disease is controlled and your medication is at ‘maintenance’ level, you should not get noticeably more infections than anyone else. But because you are immunosuppressed it can be harder to get over them. For example, a bad cold may be a lot worse for someone on immunosuppressives and may take a lot longer to get better.

You should try not to get dirt in any erosions. You should see your GP if you’re worried about how something is healing.

Coming into contact with chicken pox can be dangerous if you have not had it before because it is more severe in immunosuppressed people. If this happens, and you start to feel ill, go to your GP immediately.

Q12: The prescriptions I need are so expensive. Is there a way to get the medicines cheaper?

Some people may be eligible for free prescriptions (e.g. if they are receiving Income Support). For those who are not eligible, you can save money by buying a three month or one year Prescription Pre-payment Certificate. You can pay for a 12-month PPC in 10 direct debit instalments. For more information contact the PPA:

By phone:

For general queries, before applying for Certificates: 0845 850 0030

For specific queries, after applying for / receiving Certificates: 0845 601 8076 or look at http://www.ppa.org.uk/ppa/ppc_intro.asp

Q13: I’ve got pemphigus vulgaris in the mouth. The hospital suggested I shouldn’t eat crisps or crusty bread. Why is this? Is there other food or drink I shouldn’t have?

Pemphigus vulgaris makes the inside of the mouth very delicate and many people find that eating foods with sharp edges, like crisps or crusty bread, can tear open healing erosions or just be very painful. Food and drink affect different people differently, but it’s fairly common for people to find that highly spiced food like curries and foods containing chilli are painful when mouth erosions are present. Some people also find that acidic foods, like tomatoes or oranges, are painful. The same thing can be true of drinks; sometimes people find red wine or acidic fruit juices (e.g. cranberry or orange) hurt. There’s no general rule, you’ll have to try things out in small quantities to judge if they are painful for you. Nutritional factors may also be aggravating factors in pemphigus vulgaris flare-ups (see ‘Does anything make pemphigus vulgaris worse?’).

Q14. When I brush my teeth, my gums bleed and it is very sore. What can I do?

This is a very common problem but keeping teeth clean is very important. Gum disease and rotting teeth can occur when you have PV, simply because it can be difficult to keep the teeth clean. Nevertheless, there are ways to help avoid these problems. Buy a soft, child’s toothbrush and toothpaste for sensitive teeth (you may find strong minty toothpaste very painful). Fifteen minutes before brushing, swish some anaesthetic mouthwash e.g. Difflam, around the mouth for a minute or two to help reduce the pain of brushing. Ultrasonic and electric toothbrushes are very good for preventing plaque build-up, so you might want to use one if the discomfort is manageable. Avoid sweet, sugary foods that increase plaque formation. It’s important to see your dentist and dental hygienist regularly because they will be able to help you keep your teeth in good condition. They might recommend an antiseptic mouthwash, like Corsodyl, which helps keep the gums in good condition. Your teeth may become temporarily stained brown, but the staining will fade in time and is preferable to possible gum and tooth damage which could be irreversible.

Q15: My doctors sometimes talk about blisters and sometimes say erosion, lesion or ulcer. But it all seems to mean the same. Can you explain?

There is no general rule about what word to use.

A dermatologist’s definition of both ‘erosion’ and ‘ulcer’ would be: “an area of skin in which the top layer is missing. The undersurface of the skin is visible and looks red, raw and sore, like a burn”. But ulcers go deeper than erosions so, strictly speaking, erosion is the medically correct word to use in PV. A‘ lesion’ is a word used to describe any abnormal area of skin, therefore the blisters and erosions that occur in PV can both be called lesions.

So you can see that from the point of view of a patient, there’s no real difference between the three words.

Sometimes different words are used because they’re describing what’s happening at different stages in the process.

What happens first of all is that the action of autoantibodies causes a fragile blister to form. Because the blisters are so fragile they very quickly burst – particularly in the mouth – and what is left is an eroded area of skin (that looks as if the top layer has been scraped off). This is sometimes called an erosion, a lesion, a burn-like lesion, or an ulcer.

When doctors talk about a pemphigus ulcer they don’t mean the ordinary sort of mouth ulcer that lots of people get and which will go away of its own accord.

You will probably find that if you talk to anyone except a pemphigus specialist about “mouth ulcers” or “blisters”, they won’t understand the pain and seriousness of what’s involved. So lots of pemphigus patients say they communicate better with people by referring to “erosions”, “burn-like lesions”, or just “lesions”.

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