Pemphigus Vulgaris - Symptoms and Treatment

Pemphigus is a group of rare skin disorders that cause blisters of your skin or mucous membranes, such as in your mouth or on your genitals. It is the most common subtype of pemphigus, accounting for 70% of all pemphigus cases worldwide although it is extremely rare in New Zealand (about one case per million). The other two main subtypes of pemphigus are pemphigus foliaceus and paraneoplastic pemphigus. Pemphigus usually occurs in people between the ages of 30 and 60, but it also can affect children and older adults. Men and women develop pemphigus equally, and it affects all races and cultures. However, pemphigus tends to be more common in people of Middle Eastern or Jewish descent. Usually a chronic condition, pemphigus is best controlled by early diagnosis and treatment, which may include medications or treatments similar to those used for severe burns. Left untreated, pemphigus may be fatal.

Pemphigus is an autoimmune disorder, which is generally caused by auto antibodies directed against critical adhesion molecules of squamous epithelial cells, the desmogleins. The exact cause of the production of these antibodies against the body's own tissues is not known to anyone. Affected individuals have high concentrations of antibodies produced by the immune system. These bind to a specific protein in the skin (desmoglein 3 and sometimes desmoglein 1), causing interference with the skin's normal function. The cells no longer stick together and become separated from each other. This causes the burn-like lesions or blisters that are typical of PV. Symptoms often occur first in the mouth, but lesions can cover a significant area of skin. The disease is non-infectious. Although it is known that desmoglein antibodies cause PV, it is not known why people develop these autoantibodies in the first place. Genetic factors are believed to be important but it is likely that there are other factors which help trigger PV.

Causes of Pemphigus Vulgaris

Common Causes of Pemphigus Vulgaris :

• Pemphigus vulgaris is an autoimmune disorder which generally causes blistering. This disease is not contagious that is not transferable by touch.The disease may be a result of reactions to medications but this rarely occurs. This may be an adverse reaction to thiol groups contained by most of the drugs and the Alliums family, which includes garlic, onions and leeks.
• A history of having autoimmune diseases, such as myasthenia gravis , lupus , or thymoma
• Regular use of certain drugs, including :-
• Piroxicam.
• Penicillamine.
• Phenobarbital.

Symptoms of Pemphigus Vulgaris

Some common Symptoms of Pemphigus Vulgaris :

• Rash.
• Mouth lesions.
• Bullae on skin .
• Painful lesions.
• Blisters.
• Mouth or skin ulcers.
• Located on the scalp, trunk, or other skin areas.
• Blistering, loose.

Treatment of Pemphigus Vulgaris

• Treatment which is generally used is the administration of oral steroids, especially prednisone.
• Patient should be closely monitored by doctors because the drugs may have severe side effects.
• Doctors uses some immunosuppressive drugs such as Azathioprine , Dapsone , Tetracyclines,Cyclophosphamide, methotrexate.
• Tylenol or other painkillers (analgesic) may be used.
• Antibiotics intravenously or through the veins (IV) if lesions are infected.
• Replace fluids if patient dehydrated.
• Benadryl solution or Dyclonine candy (for kids) can help with mouth pain.

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