Bullous Pemphigoid

Bullous pemphigoid is an autoimmune disease that causes eruptions of blisters.

Bullous pemphigoid is an uncommon skin disease characterized by tense blisters on the surface of the skin. Occasionally, the inner lining tissue of the mouth, nasal passages, or conjunctivae of the eyes (mucous membrane tissue) can be involved.

• The condition is caused by antibodies and inflammation abnormally accumulating in a particular layer of the skin or mucous membranes. This layer of tissue is called the "basement membrane."
• These antibodies (immunoglobulins) bind to proteins in the basement membrane called hemidesmosomal BP antigens and this attracts cells of inflammation.
• The mucous membrane disease is also referred to separately as mucous membrane pemphigoid.

A majority of those affected by bullous pemphigoid are 50 years of age or older. While the cause is unknown, it is felt by some that an aging immune system may become activated in certain individuals with a genetic predisposition to develop bullous pemphigoid.

Symptoms of bullous pemphigoid include intense itching and a burning sensation in the skin.

When the mucous membranes of the mouth are affected, it can cause the following:

• Pain
• Burning
• Peeling away of affected inner lining tissues
• Sensitivity to acidic foods

Eating can be difficult, and involvement in the deeper areas of the throat can cause coughing. Involvement of the inner nose can cause nosebleeds.

The disease typically worsens and improves over time.

No. Bullous pemphigoid cannot be transmitted from one person to another.

Bullous pemphigoid is diagnosed and treated by dermatologists.

Bullous pemphigoid is often diagnosed by clinical examination and is confirmed by the results of a biopsy of involved tissue. The biopsy with traditional pathology evaluation can demonstrate inflammation of the affected skin levels.

Additional testing of the biopsy specimen for antibody immune deposits can reveal the abnormal antibodies located in the basement membrane layer of skin or mucous membrane tissue. Blood testing for circulating basement membrane antibodies can also be helpful.

Bullous pemphigoid-like condition can sometimes be associated with other illnesses, including systemic lupus erythematosus (SLE or lupus) and cancer.

Treatment of bullous pemphigoid is individualized and depends on the location and severity of the disease.

Bullous pemphigoid can be chronic and mild without substantially disturbing the general health of affected individuals. It can also significantly affect daily lives in its more severe forms.

• Mild bullous pemphigoid can resolve with topical prescription corticosteroid creams but sometimes requires high doses of steroids taken internally.
• Severe bullous pemphigoid can also require immune-suppression drugs such as azathioprine (Imuran), mycophenolate (Cellcept), and methotrexate (Rheumatrex). The tetracycline derivatives such as minocycline or doxycycline have also been used as an option to reduce inflammation. Other treatments that have been used for severe disease include intravenous immunoglobulin infusions, typically given monthly.

Research has indicated that large quantities of high-potency topical corticosteroids applied to the body surface are safer in controlling localized bullous pemphigoid than oral corticosteroids. It was felt by the researchers that topical corticosteroids should now be the treatment of choice for bullous pemphigoid, particularly when the disease is not extensive.

No. There are no home remedies for bullous pemphigoid other than preventing skin irritation and infection from scratching.

The outlook for bullous pemphigoid is variable. As described above, the symptoms tend to wax and wane. In its most severe form, it can be fatal without treatment, especially if involving the airways and pharynx.

No. There is no prevention for bullous pemphigoid.