Pemphigus vulgaris

Pemphigus vulgaris

What is pemphigus vulgaris?

Pemphigus vulgaris is a rare autoimmune skin disease and affects around 3 people per 100,000 population. Pemphigus vulgaris may cause severe blistering of the skin and the mucous membranes lining the mouth, nose, throat, eyes and genital area. Blisters develop in the upper layer of the skin and have a thin and fragile outer surface that breaks away easily leaving raw areas (erosions) that can be extensive and painful.

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What does it look like?

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Main body location

Eyes, Face, Genitalia, Mouth, Neck / Throat, Nose

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Can it appear anywhere?

Yes

What causes pemphigus vulgaris?

Our immune system makes antibodies to fight infection. Normally these antibodies do not attack our own body. However, in an autoimmune disease, such as pemphigus vulgaris, the immune system makes antibodies (autoantibodies) that work against tissues in the body.

  • The autoantibodies in pemphigus vulgaris attack proteins called desmogleins. These proteins are present on the cells in the outer layer of skin (the epidermis) and form the “glue” that holds the cells of the skin together.
  • When the autoantibodies formed in pemphigus vulgaris attack the desmoglein proteins, the cells in the skin and mucous membranes no longer hold together and separate. This causes blisters and erosions that are typical of pemphigus vulgaris.
  • Although it is known that antibodies to desmoglein cause pemphigus vulgaris, it is still not clear why some people develop these autoantibodies.

Pemphigus vulgaris affects males and females equally. It can start at any age but is most common in adults between 40-60 years old. It affects people of all races but is more common in some people, for example, people of Jewish or Indian origin. Pemphigus vulgaris is not an infection and therefore cannot be caught or passed on to anyone else.

Is pemphigus vulgaris hereditary?

Pemphigus vulgaris does not pass from generation to generation. However, genetic factors are thought to be important in combination with other factors.

What are the symptoms of pemphigus vulgaris?

Skin erosions (sores) are painful and can affect quality of life including disturbing sleep.  Mouth and throat lesions can interfere with eating and drinking and so cause weight loss. Pemphigus vulgaris can also affect other mucous membranes such as the genital area, leading to painful sexual intercourse, urination and bowel movements, the nose causing stuffiness and blood-stained crusts; and the conjunctiva of the eyes causing sore, red eyes (requiring assessment by an ophthalmologist).

The most common area to be affected is the inside of the mouth. This is most commonly the first area to be affected, and skin lesions often appear later. Other areas, as described above, are less commonly affected.

What does pemphigus vulgaris look like?

The skin:

  • The skin lesions start as thin-walled fragile blisters (collections of clear fluid within the skin) that burst easily, leaving raw areas known as erosions.
  • Erosions are sore, burn-like areas that can ooze fluid or become crusty and infected and when they heal, the skin may be discoloured.

The mouth:

  • Blisters in the mouth quickly burst to form erosions. There may be one or two, or several, that join together.

Can pemphigus vulgaris be cured?

Pemphigus vulgaris is a long-lasting (chronic) disease and will not usually settle without treatment. There will be occasions when it flares up and other occasions when it improves. Currently, there is no way of predicting when flares will occur or how severe they will be. Treatment is aimed at controlling the disease and preventing flares rather than a cure.

How is pemphigus vulgaris diagnosed?

  • It is advisable your general practitioner refers you to a dermatologist or an oral medicine specialist. These specialists will be able to make a provisional diagnosis of pemphigus vulgaris by examining the affected area of skin and in the mouth.
  • biopsy of a blister may be taken to confirm the diagnosis. The biopsy sample will be processed in the laboratory and examined under the microscope. Part of the biopsy sample will be examined by a technique known as direct immunofluorescenceto demonstrate the presence of pemphigus vulgaris autoantibodies in the skin.
  • Pemphigus vulgaris autoantibodies can be measured in the blood (indirect immunofluorescence). Measurement of autoantibodies in the blood is a useful test to monitor how active the disease is and can help determine if a change of treatment is needed.

How can pemphigus vulgaris be treated?

Self-care (What can I do?)

  • Keep all of your appointments with your doctor and hospital specialist.
  • Take medicines as advised by your specialist and never stop taking corticosteroids suddenly without talking to your doctor.
  • If you have mouth erosions avoid eating spicy, acidic or hard foods.
  • See your dentist or hygienist regularly for check-ups.
  • Tell your doctor if your nose, throat or genital skin are affected.

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This information is provided by the British Association of Dermatologists.


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