What Is Pemphigus?

Pemphigus is not one condition, but many rare skin disorders that causes blisters to appear on the skin. The main sites for this blistering is on the genitals and around the mouth, although it may appear in many other places. Pemphigus affects all age groups, but it is mainly seen in older people. Although it affects people of different races, it mainly affects people of Middle Eastern and Jewish descent.

Causes

• Researchers do not know exactly what causes pemphigus, but have found that it is an autoimmune disorder. Normally your body produces antibodies that get rid of harmful invaders such as bacteria and viruses, but with pemphigus your body's antibodies attack healthy skin cells, mucous membranes and proteins named desmogleins. Desmogleins proteins bind the skin's cells together. Pemphigus can also come from medication side effects. The ones most responsible are blood pressure medication and chelating agents. This form of pemphigus disappears after the medication is stopped. Other things that can cause pemphigus are additional antibodies that are seen in some people who have leukemia and lymphoma.

Symptoms

• Most symptoms of pemphigus are outward, causing blisters and open sores. There are different signs and symptoms depending on what type of pemphigus you have. Pemphigus vulgaris is the most common form of pemphigus causing blistering in the mouth and skin. These blisters can also form on the genitals. The second form of pemphigus called pemphigus foliaceus causes blisters on the face and scalp, and then progresses to form blisters on the chest and back. These blisters are usually crusty. The third type of pemphigus is pararaneoplastic pemphigus. Symptoms of this condition are painful mouth sores, sores on the lips and in the esophagus, as well as skin lesions. In rare cases, they can cause lesions in your lungs causing progressive lung disease.

Diagnosing and Testing

• Pemphigus is difficult to diagnose because blisters can be a symptom of many other skin conditions. The way pemphigus is diagnosed is by way of taking medical and family history, in addition to looking for blisters in the mouth and skin. Pemphigus causes the top layers of skin to easily separate from the lower levels. Other tests that may show signs of pemphigus are a skin biopsy and blood test.

Treatment

• Treatments mainly aim at decreasing symptoms and preventing complications. Once pemphigus becomes widespread, it is more difficult to treat. If pemphigus is mild (not widespread), corticosteroids are given as a first course of treatment. Your physician may feel that you need to combine the corticosteroids with other medications such as immunosuppressants, antifungal medications or antibiotics.

Warnings

• A few complications may arise from the pemphigus. The main one is infection of the skin stemming from blisters and open lesions. This infection may spread to the bloodstream causing sepsis (blood poisoning). Sepsis can become fatal. According to Mayo Clinic, respiratory problems may cause fatality as a complication of paraneoplastic pemphigus 90 percent of the time.