Pemphigus vulgaris is an autoimmune skin disorder characterized by blistering of the skin and mucous membrane. It is the most common subtype of pemphigus, accounting for 70% of all pemphigus cases worldwide. PV is rare – between one and five people in a million develop PV each year in the UK. Most cases develop in people aged over 50. It is very rare in children. Men and women are equally affected. It is more common in certain racial groups. Pemphigus involves blistering of the outer (epidermal) layer of the skin and mucous membranes. It is an autoimmune disorder in which the immune system produces antibodies against specific proteins in the skin and mucous membrane. These antibodies produce a reaction that leads to a separation of epidermal cells.
The exact cause of the development of antibodies against the body’s own tissues is unknown. People with PV have a slightly higher than average chance of also having other auto-immune diseases such as pernicious anaemia, rheumatoid arthritis, and vitiligo. About one-half of the cases of pemphigus vulgaris begin with blisters in the mouth, followed by skin blisters. The blisters ( bullae ) are relatively asymptomatic.Pemphigus is often associated with cancer, and other autoimmune diseases, such as myasthenia gravis. Several months’ later blisters on the skin may develop or in some cases mucosal lesions are the only manifestation of the disease. The most common mucosal area affected is the inside of the mouth but others.
Severe cases of pemphigus are treated similarly to severe burns. Oral corticosteroids are the mainstay of medical treatment for controlling the disease. An immunosuppressant such as azothiaprine, ciclosporin, etc, is also commonly advised. These medicines work by suppressing the immune system. A steroid cream is sometimes used on the skin blisters in addition to other treatments. Other immune suppressive drugs are used to minimise steroid use. Plasmapheresis is a process whereby antibody-containing plasma is removed from the blood and replaced with intravenous fluids or donated plasma. Some antibiotics are also effective, particularly minocycline and doxycycline. Intravenous immunoglobulin (IVIg) is occasionally used and immune phoresis procedures are also a possible treatment.
Pemphigus Vulgari Treatment and Prevention Tips
1. Intravenous fluids, electrolytes, and proteins may be required.
2. Anesthetic mouth lozenges may reduce the pain of mild to moderate mouth ulcers.
3. Antibiotics and antifungal medications may be appropriate to control or prevent infections.
4. Steroids and other immune suppressants can usually control the disease.
5. Oral corticosteroids are the mainstay of medical treatment for controlling the disease.