Pemphigus vulgaris (PV) is a rare and serious condition that causes painful blisters to develop on the skin as well as inside the mouth, nose, throat, anus and genitals.
The blisters are fragile and can easily burst open leaving painful areas of raw, unhealed skin.
The blisters can fade and flare up later and it is impossible to predict how severe a flare-up might be.
Read more about the symptoms of pemphigus vulgaris.
PV is what is known as an autoimmune condition. This means the immune system (the body’s defence against infection) malfunctions and starts attacking healthy tissue.
In cases of PV the immune system attacks cells found in a deep layer of skin as well as the cells found in the mucous membrane (the protective lining of the mouth, nostrils, throat, genitals and anus). This causes blisters to form in the affected tissue.
It is unclear why the immune system goes wrong in this way. PV could be caused by a combination of both genetic and environmental factors. What is known is that PV is not contagious and cannot be passed from one person to another.
Read more about the possible causes of pemphigus vulgaris.
Treating pemphigus vulgaris
There is currently no cure for PV, although the symptoms respond well to high doses of steroid medication (corticosteroids).
Using a high dose of steroids over a long period of time can cause serious side effects. So when the symptoms have cleared, low doses are used in combination with other medicine. This is known as steroid-sparing therapy, and it prevents some of the dangerous side effects associated with long-term steroid use.
Recent research suggests that a type of medication called rituximab may be an effective treatment in severe cases that fail to respond to conventional treatment.
Read more about treating pemphigus vulgaris.
Complications
A common complication of PV is the blisters becoming infected. In some cases infection can spread quickly into the blood (sepsis), affecting the whole body, which can be life-threatening.
Other complications include the side effects associated with steroid and immunosuppressant use.
Read more about the complications of pemphigus vulgaris.
Who is affected
PV is a rare condition. It is estimated that each year in England only 1 in every 145,000 people will be newly diagnosed with PV.
Most cases first develop in older adults aged 50-60, although PV can affect people of all ages, including children. Both sexes are equally affected.
PV is slightly more common in people of Jewish, Indian and Mediterranean descent.
Outlook
The outlook for people with PV has improved significantly since the introduction of steroids, although the complications associated with the condition can be life-threatening.
Most people with PV experience flare-ups when symptoms are particularly bad and then periods when they experience no or very few symptoms (this is known as remission). In some people the symptoms may go away permanently (complete remission).
