Pemphigus Vulgaris
This page covers the following:
Introduction
Pemphigus Vulgaris (PV) is a rare autoimmune blistering disease (AIBD) with up to 2.7 cases per 100,000 people diagnosed per year. The disease can appear suddenly, causing severe blistering of the skin and the mucous membranes lining the mouth, nose, throat and genitals. Because the autoantibodies that cause PV affect the upper layers of the skin, blisters appear as fluid-filled sacs developing on your skin and mucosa which are very thin and fragile and break easily leaving raw areas (erosions) that can be extensive and painful.
PV does not go away by itself, and always needs treatment by a specialist, normally a dermatologist.
PV affects both males and females equally. It can start at any age but is most common in adults usually between 50-60 years old. It affects people of all races but is more common in people with Middle Eastern, Central Asian and Ashkenazi Jewish heritage.
The blisters and erosions usually start in your mouth, and appear later on your skin. In some patients, however, the skin is affected first. Other sites affected include the conjunctiva, oesophagus, labia, vagina, cervix, penis, urethra and anus.
The raw erosions of PV are painful and often take a long time to heal. As well as being very sore, similar to burns-they can ooze fluid or become crusty and infected and when they heal, the skin may be discoloured. Some PV sufferers report that they thought they had been bitten by insects when the disease first manifested on the skin, as the blisters sometimes begin as small red itchy bumps.
Blisters and erosions in your mouth can interfere with eating and drinking and can lead to weight loss. Most patients get erosions in their mouth at some time but some never get blisters or erosions on their skin and an initial diagnosis often follows a trip to your dentist. When PV affects the conjunctiva of your eyes, an ophthalmologist should be consulted.
Sites often overlooked include around your nails (manifest as painful, red, and swollen), pharynx and larynx (pain on swallowing and hoarseness), and your nasal cavity (nasal congestion and a bloody mucous discharge, particularly noticeable upon blowing your nose in the morning). Blisters in the larynx can cause hoarseness when talking, and the airway could be impacted which can require emergency treatment. As many as 49% of patients were shown to have laryngeal and nasal involvement.
How is pemphigus vulgaris diagnosed?
Often it will be your GP or dentist who will make the initial visual diagnosis and offer a referral to a specialist depending on the part of the body affected. The most common departments are Dermatology, Ear, Nose and Throat (ENT), Maxillo-Facial (Max-Fax) or to a dental hospital.
A biopsy of a blister is usually taken to confirm the diagnosis. The biopsy sample will be processed in the laboratory and examined under a microscope. Part of the biopsy sample will be examined using a technique known as direct immunofluorescence to demonstrate the presence of PV autoantibodies in the skin. It is not unusual to have more than one biopsy taken, especially if both the skin and mucous membranes are involved.
Pemphigus vulgaris autoantibodies measured in the blood (by a specialist technique known as ELISA) can be a useful test to monitor how active the disease is and can help determine if a change of treatment is appropriate.
How long will my pemphigus vulgaris last?
PV is a chronic autoimmune disease with no known cure. Suppressing your immune system is necessary to keep the disease under control and make remission possible. The initial goal of treatment is to clear existing blisters and help prevent relapses. Treatment typically depends on the severity and stage of your disease, with early diagnosis and treatment beneficial to getting your condition under control. Unfortunately, this disease can have unpredictable episodes where it flares for a variety of reasons such as stress or illness in which case treatment may need to be adjusted.
Before the advent of corticosteroids, PV had a high fatality rate, with approximately 70+% of patients dying within a year. Nowadays, it is very rarely a cause of death. With treatment, lesions can heal normally without scarring and the hyperpigmentation associated with pemphigus usually resolves after several months-years. Most patients treated for PV will enter a partial remission within 2 to 5 years.
How is pemphigus vulgaris treated?
The key to bringing PV under control is through the suppression of your immune system to stop the recurrence of flares. This can only be achieved by a range of drugs, and it can take time and the use of different drugs and/or a combination of drugs to find the treatment that works best for you. No two patients with PV are the same. The aim of the treatment is to prevent new blisters from forming and to heal broken areas of skin. It can take about 2 to 3 weeks to stop new blisters developing, and approximately 6 to 8 weeks for healing to occur. Once your disease is controlled, the dose of the medication can be reduced slowly to the lowest effective level. Treatment usually starts with a corticosteroid, usually Prednisolone, and is typically combined with a ‘steroid-sparing’ immunosuppressive drug (see below).
What drugs might I be offered to reduce the impact of pemphigus vulgaris?
In moderate to severe cases, oral corticosteroids (such as Prednisolone) are usually prescribed, often in high doses, to stop the blistering and reduce inflammation. They can also be prescribed in conjunction with other drugs, especially in more severe cases. Corticosteroids are extremely effective and can work much quickly than other treatments which can be vital, especially if your airway is affected. Once your disease is under control and no new blisters have formed, then the slow process of tapering off the steroids can begin, and an alternative drug can be prescribed to replace the steroids, if they haven’t been prescribed initially. These are called “steroid sparing drugs” as they should enable gradual reduction and cessation of oral corticosteroids. Steroid-sparing drugs typically a better long term safety profile compared to steroids but they have their own side effects, and it sometimes takes a while to find a treatment that works.
It is dangerous to stop oral corticosteroid treatment suddenly because your body may have become highly dependent on this medication. This is because your body may have stopped making its own natural corticosteroids. If you have concerns about your steroid treatment, please consult your doctor. For more information see our page on steroids.
The steroid-sparing immunosuppressant drugs most commonly prescribed for PV include Mycophenolate, Azathioprine, and Cyclophosphamide. For more information see our page on other medications.
The immunosuppressant Rituximab, given as an infusion, has been shown to be highly effective in bringing PV into remission. In England and Scotland, this is only currently offered after oral steroids combined with another drug have failed to adequately control the disease. Therefore, Rituximab is referred to as a third-line treatment.
Topical treatments will also be necessary to help the lesions heal both on the skin and the mucus membranes. A steroid cream or ointment is usually applied to skin blisters and erosions so that the dose of steroid tablets can be kept lower. Mouth blisters and erosions may be treated with steroid mouthwashes or sprays and mouthwashes containing antiseptic or local anaesthetic. For more information see our page on topical steroids.
Where can I find out more information?
We recommend the British Association of Dermatologists Patient Leaflet, the New Zealand DermNetNZ website and the International Pemphigus and Pemphigus Foundation.