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Diseases and Conditions
Bullous pemphigoid
From MayoClinic.com
Special to CNN.com

Introduction

Bullous pemphigoid is a rare, chronic condition in which fluid-filled blisters (bullae) erupt on the surface of your skin, usually on your arms and legs. The cause of bullous pemphigoid is unknown, but it may be related to a disorder of the immune system.

Bullous pemphigoid typically occurs in older adults. The condition is seldom life- threatening, but the medications used to treat bullous pemphigoid can cause complications. Without treatment, bullous pemphigoid may persist, with periods of remission and flare-ups, for many years.

Signs and symptoms

Signs and symptoms of bullous pemphigoid range from mild to severe. Some people might only notice a slight redness and irritation on their skin, while others may experience multiple blisters. The bullae typically develop on your arms, legs or trunk but can also occur in your mouth or other mucous membranes.

The blisters appear as firm, fluid-filled sacs at least 1 centimeter in diameter. They may weep or appear crusty. In some cases, the blisters may form painful, open sores on the surface of your skin.

Other signs and symptoms may include:

  • Itching
  • Rashes
  • Mouth sores
  • Bleeding gums
  • General ill feeling

Causes

Bullous pemphigoid is a disorder in which antibodies produced by your immune system attack a thin layer of connective tissue in your skin (basement membrane). It's not certain what causes antibodies to attack healthy tissue in your body. If the delicate basement membrane becomes inflamed, fluid-filled blisters can form.

Risk factors

Bullous pemphigoid is most likely to occur in people who are 60 years of age and older. The condition is uncommon in young people.

When to seek medical advice

If you develop an unexplained blistering on your skin or an itchy rash, talk to your doctor. Bullous pemphigoid is usually evaluated by a doctor who has special training in diagnosing and treating skin conditions (dermatologist).

Screening and diagnosis

The characteristic fluid-filled blisters are often the first indication that you may have bullous pemphigoid. Usually, your doctor will take a sample of your skin (biopsy) and conduct special tests under a microscope. Your doctor may also order a blood test to confirm the presence of antibodies associated with bullous pemphigoid.

Complications

The most common complications of bullous pemphigoid arise from the medications used to treat the condition. Drugs called immunosuppressants help treat bullous pemphigoid, but they also subdue your immune system, which may increase your likelihood of developing certain infections and cancers. Taking corticosteroids over long periods of time may increase your risk of osteoporosis and bone fractures.

Bullous pemphigoid can also affect your quality of life. Blisters appearing in your mouth may make it more difficult for you to eat. You may find that it's tricky to complete your daily activities if you have ruptured blisters or blisters on the palms of your hands or soles of your feet. Also, bullous pemphigoid occurring in the eyes can lead to scarring.

Treatment

The goal of treating bullous pemphigoid is usually to reduce inflammation — thereby easing the symptoms — and suppressing the autoimmune response in your skin.

  • Corticosteroids. Corticosteroids help relieve inflammation. You can take corticosteroids as a pill (oral) or as a cream that you spread on the surface of your skin (topical). Most people with bullous pemphigoid need to take oral corticosteroids at least in the beginning of therapy.
  • Immunosuppressants. These drugs help calm the autoimmune response in your skin, which reduces inflammation.
  • Corticosteroid-sparing agents. Drugs that help reduce the dosage or the need for continued use of corticosteroids are referred to as "corticosteroid-sparing."

With therapy, bullous pemphigoid usually disappears within one and a half to five years. However, left untreated, the condition may disappear spontaneously after a couple months or persist for years.

Prevention

There's no prevention for bullous pemphigoid. Certain drugs provoke a reaction that produces blisters similar to those of bullous pemphigoid in some people. If you notice any skin problems after beginning a medication, talk to your doctor.

Self-care

If you have bullous pemphigoid, you can help take care of your condition in several ways. Here are a few ideas:

  • Watch your diet. If you have blisters in your mouth, avoid eating hard and crunchy foods, such as chips and raw fruits and vegetables.
  • Avoid injury. Your skin is fragile, both from bullous pemphigoid and the medication used to treat it, so take care to prevent trauma. If you accidentally break a blister on your skin, cover it with a dry, sterile dressing to protect it from infection as it heals.
  • Take necessary supplements. If you're taking oral corticosteroids for longer than a month, talk to your doctor about taking calcium and vitamin D supplements to help prevent osteoporosis.
  • Prepare for the sun. At least 30 minutes before you head outdoors — winter or summer — generously apply sunscreen with a sun protection factor (SPF) of 15 or greater.

June 09, 2006

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