Definition

Bullous pemphigoid is an autoimmune disorder that causes blistering of the skin.

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Causes

The immune system normally attacks foreign matter to prevent or stop infections. When the immune system attacks healthy tissue it is called an autoimmune disorder. In bullous pemphigoid, the immune system attacks tissue just below the top layer of skin.

The exact cause of bullous pemphigoid or autoimmune disorders in general is not clear. Some bullous pemphigoid may be associated with other immune diseases such as diabetes or rheumatoid arthritis.

Risk Factors

Bullous pemphigoid is more common in people aged 60 years and older.

In a small number of cases bullous pemphigoid may occur after:

• Neurological diseases, such as epilepsy
• Certain medication, such as penicillin or diuretics
• Burns or trauma
• UV radiation exposure
• Radiation therapy

Symptoms

Severe itching of the skin is often the first sign. Eventually an eczema-like or hive-like rash and blistering will develop. Blisters are usually clear and are most commonly seen on the arms, legs, abdomen, and mouth, usually along creases in the skin. The blisters can break and form ulcers.

It may be a few years between the start of itching and the appearance of a rash.

Diagnosis

You will be asked about your symptoms and medical history. A physical exam will be done. You may be referred to a skin specialist.

Tests may be done to help distinguish bullous pemphigoid from other conditions with similar symptoms. Tests may include:

• Blood tests
• Skin biopsy —a sample of the affected tissue will be removed for testing

Treatment

Bullous pemphigoid responds well to treatment and usually resolves completely within 5 years. Talk with your doctor about the best treatment plan for you. Options include:

Certain medications may help lower the immune system and decrease damage to the skin. Medication options may include:

• Steroids—(topical, oral, or injected) to reduce the immune system reaction
• Immunosuppressant medication—(oral or injected) to lower the immune system reaction in those that do not respond well to steroids
• Antibiotics—(topical) to treat infection and reduce inflammation in those who cannot take steroids or have to take reduced doses of steroids; they may be used with steroid creams

Antibiotics or antiseptic medications may also be used to prevent or treat infections in broken skin.

Blisters will be left intact when possible. However, blisters that interfere with daily tasks may be drained.

Proper skin care will decrease the risk of infection in damaged skin.

Prevention

Since the cause is not clear, there is no known way to prevent bullous pemphigoid.