Pemphigus

Pemphigus, derived from the Greek word pemphix meaning bubble or blister – is a medical term used to describe a group of autoimmune related blistering skin diseases. This family of blistering skin disorders usually affects the mouth and other mucous surfaces of the body, but can include other areas of the skin as well.

There are generally three types of the disease, and they include pemphigus vulgaris (most commonly diagnosed - referred to as PV), pemphigus foliaceus (PF) and paraneoplastic pemphigus (the most serious of the three diseases).

Symptoms

Symptoms and severity can vary by disease type, but all three are generally characterized by the presence of blisters that eventually break and become open sores (ulcers).

• Pemphigus Vulgaris

  This variety of the disease is the most common, usually in people 40 – 60 years old. Blisters usually start in the mouth, and can later erupt over other mucous areas of the skin (i.e. genitals) or other parts of the body. The blisters after breaking open - create festering, painful sores in the mouth that can make eating very difficult. In severe cases, the entire lining of the mouth can be affected.

  Subsequent general blistering on other parts of the skin can become widespread. As the blisters rupture, the resulting open sores become susceptible to secondary bacterial infections. The blisters are usually not itchy.

  

• Pemphigus Foliaceus

  PF does not usually affect the mouth and other mucous skin areas like PV. Instead, the blisters usually form on the face and scalp that can later spread to the chest and back. Not usually painful (like PV) – the blisters can however become very itchy. Often this condition is misdiagnosed as general dermatitis or eczema.

• Paraneoplastic Pemphigus

  Although the least common of all three diseases in this family, it is generally considered the most serious and/or severe. This skin disorder is generally attributed to a complication of lymphoma or leukemia. The appearance of this disease may precede the diagnosis of a malignancy and can therefore alert a physician to the possible presence of a malignancy. Painful blisters (then sores) develop on the mouth, lips and esophagus.

  In some cases, lesions can also develop on the lungs leading to bronchiolitis obliterans with corresponding difficulty in breathing (dyspnea). Complete removal or cure of the tumor may improve skin disease symptoms, but any damage to the lungs is usually irreversible.

Causes

Pemphigus Vulgaris - Genetic predisposition to immune system dysfunction remains the primary factor in the cause of this disease. In addition, the appearance of other autoimmune diseases, particularly myasthenia gravis (chronic disorder characterized by muscle weakness and fatigue) or thymoma (a tumor of the thymus) increases the likelihood of developing this disease.

Pemphigus Foliaceus may be precipitated by the use of certain drugs (i.e. penicillamine, bucillamine, NSAIDs). Intense UV exposure or burns to the skin are also thought to contribute to the development of this disease. Endemic PF is thought to be induced by a viral infection transmitted by insects.

Paraneoplastic Pemphigus is caused by additional antibodies that are present in the bloodstream due to cancer, usually lymphoma or leukemia.

Other contributing risk factors are age (middle-aged or older) and heritage (Mediterranean or Jewish descent) related.

Treatment

Depending upon the severity, and sometimes “type” of pemphigus, different treatment regimens are utilized. For mild cases, oral corticosteroids (e.g. prednisone) are usually the preferred choice for initial treatment. If the disease is not too widespread, a stay at the hospital may be avoided.

Due to side effects (i.e. weight gain, mood swings, elevated blood sugar, osteoporosis and increased risk of infection) associated with the administration of corticosteroids, the initial higher doses are usually reduced once symptoms lessen. Reductions in corticosteroid dosage levels can also be achieved by combining these drugs with immunosuppressants (i.e. azathioprine, methotrexate, mycophenolic acid) or cytotoxic drugs (i.e. Imuran, Cytoxan). For mild cases of PF, hydroxychloroquine or dapsone may also help to control symptoms.

For severe cases of the disease, other treatment options are available, including a stay in the hospital for widespread blistering and ulcers (you may end up in a burn center depending on the severity and the amount of skin area covered by the blisters and open sores). For tough cases of PV, rituximab (anti-CD20 antibody) had been found to be effective where other drug therapies have failed.

Intravenous gamma globulin (IVG) can be effective for some severe cases of the disease, especially paraneoplastic pemphigus. Plasmapheresis, another viable treatment option for severe cases - is a process in which antibodies are filtered from the blood with cell separator equipment.

In all cases, when secondary bacterial infections develop, antibiotics are prescribed. Antifungal medications may also be used to help limit the initial onset or spread of secondary infections.

Without treatment, pemphigus is usually fatal due to secondary infections from the open sores. However, the good news is that survivability is 90% or better when rapid and effective treatment is introduced.

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