Bullous Pemphigoid

What is bullous pemphigoid?

Bullous pemphigoid (bull-us pem-fuh-goyd) is a rare skin condition that causes itchy, hive-like welts or fluid-filled blisters.

It may affect a small area of your body or it may be widespread. Blisters may occur anywhere, but often develop on flexural areas of your skin, such as under your armpits (axilla), around your groin or on your stomach (abdomen). In some cases, blisters also form on mucous membranes, including your mouth, tongue, throat, esophagus and/or eyes.

Is bullous pemphigoid an autoimmune disease?

Yes, bullous pemphigoid is an autoimmune disease. That means bullous pemphigoid occurs when your body’s immune system attacks the layer of tissue below your top layer of skin.

Who does bullous pemphigoid affect?

Bullous pemphigoid most commonly affects people over 60 years old, but it may also appear in younger people, too. It’s more common in the Western world, and uncommon in the Far East.

Bullous pemphigoid affects men and women equally.

You may be more likely to get bullous pemphigoid if you have a neurological disorder, including:

• Dementia.
• Parkinson’s disease.
• Stroke.
• Epilepsy.
• Multiple sclerosis.

How common is bullous pemphigoid?

Bullous pemphigoid is relatively rare. There are between 2.4 and 23 cases per 1 million people in the general population. However, in people 80 years of age and older, there are between 190 and 312 cases per 1 million.

Is bullous pemphigoid fatal?

Bullous pemphigoid may be fatal if you’re in poor health or don’t seek treatment, especially if your blisters become infected.

What are the symptoms of bullous pemphigoid?

Bullous pemphigoid appears as itchy welts that look like hives or multiple, itchy blisters (bullae). It most commonly appears on your:

• Arms.
• Legs.
• Abdomen.
• Groin.
• Mouth.

The blisters typically appear along the creases of your skin. They usually aren’t painful, but they may break open and become a painful sore or ulcer.

The fluid inside of your blisters may be clear, or it may contain some blood. The skin around your blisters may look normal or discolored (red, purple, brown or slightly darker than your normal skin color).

Welts and blisters rarely result in scarring.

What causes bullous pemphigoid?

Bullous pemphigoid is an autoimmune disease. Healthcare providers and medical researchers aren’t sure why your immune system responds this way.

Certain medications may trigger bullous pemphigoid, including the diabetes medication alogliptin. Ultraviolet (UV) light and ionizing radiation may also trigger or aggravate bullous pemphigoid.

Do certain foods trigger bullous pemphigoid?

Healthcare providers and medical researchers aren’t sure if diet affects bullous pemphigoid. Some people have reported their bullous pemphigoid improved or went away after eliminating certain foods from their diet. However, there’s currently no good scientific evidence to support that certain foods trigger bullous pemphigoid.

If you have bullous pemphigoid, talk to your healthcare provider about any potential benefits of eliminating certain foods or drinks from your diet. They may recommend keeping track of what you eat and drink in a food journal. If you notice your bullous pemphigoid flares up after eating certain foods, gradually eliminate them from your diet to see if your symptoms improve.

Is bullous pemphigoid contagious?

No, bullous pemphigoid isn’t contagious. You can’t spread bullous pemphigoid to another person through skin-to-skin contact.

How is bullous pemphigoid diagnosed?

Your healthcare provider will examine your affected areas. They may ask you questions, including:

• When did your symptoms begin?
• Do your blisters have pus or blood?
• Have you had a fever?
• Have you started any new medications?

What tests will be done to diagnose bullous pemphigoid?

Your healthcare provider may recommend the following tests to confirm their diagnosis:

• Blood test. Your healthcare provider will use a small needle to draw a blood sample from a vein in your arm. They’ll send the blood sample to a laboratory for testing.
• Skin biopsy. Your healthcare provider will remove a small skin sample and send it to a laboratory for testing.

What’s the best treatment for bullous pemphigoid?

For mild cases of bullous pemphigoid, the best treatments are topical corticosteroid creams or ointments that you rub directly on the affected areas.

For moderate-to-severe cases of bullous pemphigoid, your healthcare provider may prescribe an oral corticosteroid, like prednisone, in addition to a steroid-sparing immunomodulatory agent such as dapsone, mycophenolate mofetil, azathioprine, methotrexate or chlorambucil.

If you can’t take can’t corticosteroids or other immunomodulatory agents, your healthcare provider may prescribe oral tetracycline or doxycycline pills.

For refractory cases of bullous pemphigoid, your healthcare provider may prescribe rituximab or IVIG infusions.

Are there any home remedies for bullous pemphigoid?

There aren’t any home remedies for bullous pemphigoid, but there are things you can do to make your symptoms more tolerable, including:

• Wear soft, loose-fitting clothing made out of natural fibers.
• Avoid spending a lot of time in the sun and wear sunscreen.
• Wash your sores or ulcers with antibacterial soap and water to prevent infection. Then, apply antibiotic ointment to your affected areas and wrap them in nonadhesive (doesn’t stick to your skin) bandages.
• Avoid standing or walking for long periods if you have bullous pemphigoid on your feet.
• Moisturize your skin with lotions, creams, coconut oil or shea butter oil.

If you have bullous pemphigoid in your mouth, the following tips can make your symptoms more tolerable and help prevent nutrition problems:

• Eat a diet of soft foods, such as soups, mashed foods, pudding and applesauce.
• Wait until hot foods cool down before you eat.
• Avoid crunchy or sharp foods, including chips, cereal, crusty bread and raw vegetables and fruits.
• Avoid acidic foods, including hot peppers, salsa, citrus fruits and tomatoes.
• Avoid drinking alcohol.

How soon after treatment will I feel better?

You should start to feel better a few days after starting treatment.

How can I prevent bullous pemphigoid?

Healthcare providers and medical researchers aren’t sure how to prevent bullous pemphigoid.

What can I expect if I have bullous pemphigoid?

Bullous pemphigoid usually clears up on its own after about five years. It generally responds well to appropriate treatment. You may have flare-up and times where it seems to go away (remission). During remission periods, you may stop your treatment.

If your blisters pop and become infected, you’re at risk of developing sepsis, which is a life-threatening condition.

When should I see my healthcare provider?

Contact your healthcare provider as soon as you develop unexplainable blisters.

What questions should I ask my healthcare provider?

• How can you tell that I have bullous pemphigoid?
• If I don’t have bullous pemphigoid, what other skin condition might I have?
• How can I manage my symptoms?
• What medications do you recommend for me?
• Do the medications have any side effects?
• What else should I do to improve my symptoms?
• How long will I have bullous pemphigoid?
• Should I see a dermatologist or another specialist?

What’s the difference between bullous pemphigoid and pemphigus vulgaris?

Both bullous pemphigoid and pemphigus vulgaris are autoimmune blistering diseases. Pemphigus vulgaris symptoms share some similarities to bullous pemphigoid symptoms. Pemphigus vulgaris almost always starts on the mucous membranes inside of your mouth. Very few people have involvement of the skin. Also, the blisters in pemphigus vulgaris are much more fragile than those in bullous pemphigoid.

People with pemphigus vulgaris usually have erosions if their skin is involved, not intact blisters like bullous pemphigoid people typically develop. If you have pemphigus vulgaris, the top layers of your skin easily separate from your lower layers of skin with pressure, usually from pressing or rubbing (Nikolsky sign).

While all people with bullous pemphigoid have skin involvement, only about 33% of them develop blisters in their mouth.

A note from Cleveland Clinic

Bullous pemphigoid is a rare autoimmune disease that causes blisters or sores on your skin. It may go away and come back, but it generally responds well to treatment and goes away completely after approximately five years. If you’re in poor health and your blisters pop and become infected, bullous pemphigoid may be fatal. Contact your healthcare provider right away if you notice blisters that you can’t explain.