Bullous pemphigoid

Definition

Bullous pemphigoid (BUL-us PEM-fih-goid) is a rare, persistent skin condition that usually appears as large, fluid-filled blisters (bullae) in your skin. Although anyone can develop bullous pemphigoid, it almost always affects older adults.

Bullous pemphigoid occurs when the immune system mounts an attack against a thin layer of tissue below your outer layer of skin. The reason for this abnormal immune response is unknown.

Drugs that suppress the immune system abnormalities of bullous pemphigoid improve symptoms, but they require careful monitoring for serious side effects. Bullous pemphigoid is rarely life-threatening, except in older individuals already in poor health or in people with otherwise poor immune system function.

Bullous pemphigoid

Bullous pemphigoid

People with bullous pemphigoid may develop multiple blisters. These typically develop on your arms, legs or trunk but can also occur in your mouth or other mucous membranes. ...

Symptoms

The primary feature of bullous pemphigoid is the appearance of blisters. Characteristics of the blisters include the following:

• Blisters are large, filled with fluid and don't easily rupture when touched.
• Fluid inside the blisters is usually clear but may contain some blood.
• Skin around the blisters may appear normal, reddish in people with light skin color or darker than normal in people with dark skin color.
• Blisters can appear anywhere on the body, but they are most often on the abdomen, groin, inner thighs and arms. Blisters are often located along creases or folds in the skin, such as the skin on the inner side of a joint.
• The site of a ruptured blister may be painful or sensitive.

Others symptoms include:

• Hives. Raised, red or dark patches of swollen skin (hives) may appear before the development of blisters. In some cases, hives may be the only skin lesion to develop.
• Itching. The affected areas of skin are usually very itchy.
• Mouth sores. You may develop blisters or sores in the mouth and, rarely, on other mucous membranes.

When to see a doctor
If you develop unexplained blistering, hives, itching or other skin irritation — a condition not caused, for example, by a known skin allergy or contact with poison ivy — see your doctor. Because a number of conditions can cause signs and symptoms affecting your skin, it's important to get a prompt, accurate diagnosis and appropriate care.

Bullous pemphigoid

Bullous pemphigoid

People with bullous pemphigoid may develop multiple blisters. These typically develop on your arms, legs or trunk but can also occur in your mouth or other mucous membranes. ...

Causes

The cause of bullous pemphigoid is not well understood. The blisters and other skin irritation occur because of a malfunction in the immune system.

Your body's immune system normally produces antibodies to fight bacteria, viruses or other potentially harmful foreign substances. For reasons that are not clear, the body may develop an antibody to a particular tissue in your body.

In bullous pemphigoid, the immune system produces antibodies to the skin's basement membrane, a thin layer of fibers connecting the outer layer of skin (dermis) and the next layer of skin (epidermis). These antibodies trigger inflammatory activity that produces the blisters, hives and itching of bullous pemphigoid.

Contributing factors
Bullous pemphigoid usually appears randomly with no clear factors contributing to the onset of disease. A small percentage of cases may be triggered by certain treatments, including:

• Prescription medications to treat rheumatoid arthritis or fluid retention
• Ultraviolet light therapy to treat certain skin conditions or inflammatory disorders
• Radiation therapy to treat cancer

Risk factors

Bullous pemphigoid is more likely to occur in people who are 60 years of age and older, and it's most common in people in their 80s.

Complications

Serious complications of bullous pemphigoid are more likely in older adults and in those in generally poor health. These include:

• Infections at the site of ruptured blisters
• Widespread infection in other tissues (sepsis)

Depending on the severity and location of blisters, bullous pemphigoid may disrupt typical activities of daily living. The blisters and itching may make it difficult to:

• Maintain personal hygiene
• Wear certain clothing or shoes comfortably
• Eat or drink if blisters or sores are in the mouth
• Sleep comfortably

A person may also be self-conscious or embarrassed by his or her appearance. All of these complications can affect a person's health, social interactions, physical activity and general well-being.

Preparing for your appointment

You'll likely start by seeing your primary care doctor if you have blisters, hives or other skin irritation. You may be referred, however, to a specialist in skin disorders (dermatologist).

You may want to bring a friend or relative to your appointment. This person, in addition to offering support, can write down information from your doctor or other clinic staff during the visit.

Prepare a list
You should make a list to share with your primary doctor or dermatologist. This list should include:

• The name and contact information of any physician you have seen recently or see regularly
• Prescription medications and dosages
• Over-the-counter drugs or dietary supplements you take regularly

What to expect from your doctor
Your doctor will likely ask you a number of questions. Be prepared to answer the following:

• When did the blisters, hives or other skin problems appear?
• Where are they located?
• Do the blisters, hives or other areas of your skin itch?
• Have you observed any oozing, draining of pus or bleeding?
• Have you recently started new medications?
• Have you had a fever?

Tests and diagnosis

Your doctor or dermatologist makes a diagnosis of bullous pemphigoid or another skin disorder based on the information you provide about symptoms, a careful examination of the skin abnormality and the results of laboratory tests. These tests include:

• Biopsy. Your doctor will remove a tiny piece of skin (biopsy) from one or more sites where your skin is affected. These tissue samples are stained with specially prepared antibodies and examined under a microscope by a laboratory technician. A particular profile of immune system antibodies revealed in the tissue sample can point toward a diagnosis of bullous pemphigoid.
• Blood test. A nurse or assistant may also draw a small sample of blood for a lab test. This test can identify concentrations of immune system proteins in the bloodstream that are typically elevated in people with the disorder.

Treatments and drugs

The goals of bullous pemphigoid treatment are to help the skin heal as quickly as possible and relieve itching. Your doctor will likely prescribe a combination of drugs that inhibit immune system activities that cause inflammation.

Most people experience improvement in symptoms within a few weeks, but ongoing treatment is often necessary for several years.

Corticosteroids
Corticosteroids taken as a pill (oral) are the primary treatment for bullous pemphigoid, particularly at the beginning of a treatment regimen. Some people may also be able to use a corticosteroid ointment (topical) that is applied directly to the affected skin. The side effects vary depending on whether you have oral or topical treatment.

• Oral side effects. Long-term use of oral corticosteroids can cause weakening of the bones (osteoporosis), diabetes, high blood pressure, high cholesterol, cataracts and other serious side effects.
• Topical side effects. Long-term use of topical corticosteroids can cause thinning of the skin and other skin problems.

After your skin has healed, your doctor will gradually reduce your dosage to the lowest dose possible to keep the disease under control and minimize the risk of side effects.

Immunosuppressants
Immunosuppressants are drugs that inhibit the production of your body's disease-fighting white blood cells. These drugs may be used in combination with corticosteroids to minimize the risks of corticosteroid treatment. They may also be used alone, but symptom relief may take longer to achieve.

Side effects of these drugs include an increased risk of infections. Immunosuppressants used to treat bullous pemphigoid include:

• Azathioprine (Imuran)
• Mycophenolate mofetil (CellCept)

Other anti-inflammatory drugs
Other drugs with anti-inflammatory properties that may be used alone or in combination with corticosteroids include:

• Methotrexate (Trexall), a drug often used to treat other inflammatory skin diseases and rheumatoid arthritis
• Tetracycline, an antibiotic with anti-inflammatory properties
• Nicotinamide, a byproduct of a B vitamin, taken in combination with tetracycline
• Dapsone (Aczone) and sulfapyridine, a combination of drugs with antibiotic and anti-inflammatory properties

Monitoring side effects of corticosteroids
Long-term use of corticosteroids can result in a number of serious side effects. Your doctor will monitor you closely for potential problems. He or she may adjust your dosage and prescribe treatments to manage reactions to corticosteroid treatment.

Your doctor will likely prescribe daily doses of calcium and vitamin D supplements to help prevent osteoporosis induced by the treatment. The American Academy of Rheumatology recommends the following daily doses for anyone taking oral corticosteroids for more than three months:

• 1,000 to 1,200 milligrams (mg) of calcium supplements
• 400 to 1,000 international units (IU) of vitamin D supplements

Lifestyle and home remedies

If you have bullous pemphigoid, you can help take care of your condition with the following self-care strategies:

• Avoid injury. The blisters of bullous pemphigoid and topical corticosteroids, if you're taking them, cause your skin to be fragile. If a blister on your skin breaks, cover it with a dry, sterile dressing to protect it from infection as it heals.
• Avoid sun exposure. Avoid prolonged sun exposure on any area of the skin affected by bullous pemphigoid.
• Watch what you eat. If you have blisters in your mouth, avoid eating hard and crunchy foods, such as chips and raw fruits and vegetables, because these types of foods might aggravate symptoms.