Bullous pemphigoid is a rare autoimmune disease that affects your skin. It’s a chronic disease that causes itchy, fluid-filled blisters to form all over your body. Although people of all ages, including children, can get bullous pemphigoid, it mostly affects adults over 60.
Bullous Pemphigoid Causes
Bullous pemphigoid is an autoimmune disease, which means something causes your immune system to attack your body’s cells. But researchers don’t know why this happens. If you have bullous pemphigoid, your body attacks the cells just under your epidermis, the outermost layer of your skin. This is the subepidermal area.
Some things can trigger bullous pemphigoid disease. They include:
- Some prescription drugs, such as:
- Alogliptin (Nesina), for diabetes
- Etanercept (Enbrel), an immunosuppressant
- Furosemide (Lasix), a diuretic (“water pill”)
- Penicillin, an antibiotic
- Sulfasalazine (Azulfidine), an anti-inflammatory
- Ultraviolet light therapy, which can be used to treat some skin conditions
- Radiation therapy to treat cancer
- Injury to the skin, including sunburn
- Skin infection
- Some medical conditions, such as:
How common is it?
Bullous pemphigoid disease is rare, but it’s the most common type of autoimmune subepidermal blistering disease. About 80% of all people who have subepidermal blistering have bullous pemphigoid. And although it’s rare, it’s more common in older people. There are up to 23 cases of the disease per million people overall in the U.S., but up to 312 cases per million among those over 80.
Bullous pemphigoid risk factors
As you age, your risk of developing bullous pemphigoid rises, but there are some other risk factors as well, such as having certain illness and conditions, like:
- Stroke
- Epilepsy
- Dementia
- Parkinson’s disease
- Multiple sclerosis
- Schizophrenia
- Personality disorders
Bullous Pemphigoid Symptoms
The primary symptoms for bullous pemphigoid are large, fluid-filled blisters on the skin, often in creases or skin folds. The blisters are almost a half-an-inch in size or larger and are most often on your:
- Arms
- Groin
- Legs
- Armpits
- Mouth
- Abdomen (Belly area)
Other symptoms can include:
- Itchy skin for weeks or even months before the blisters appear. The itch can be mild or intense. It also might only be in some areas, not all over.
- Red or darker skin around the blisters
- Itchy welts or a rash that might look like hives. This can last for days or weeks.
- Eczema-like rash
- “Solid” blisters that can be as large as 2 inches across. They feel tight and don’t rupture or break easily.
About 20% of people with bullous pemphigoid don’t get blisters, but they have some of the other symptoms.
There is also a subtype of bullous pemphigoid that causes blisters to appear on the palms of your hands and soles of your feet.
Bullous Pemphigoid Diagnosis
Getting a diagnosis of bullous pemphigoid is important so you can get the proper treatment.
If you have blisters, your doctor will probably suspect you have bullous pemphigoid. But if your doctor isn’t sure or you have other symptoms but no blisters, the diagnosis might come from:
- Physical examination of your skin
- Questions about your medical history and your symptoms, such as:
- When did the symptoms begin?
- Are you taking any medications?
- Have you had a fever?
- When the blisters pop, do you see blood or pus?
- Have you had any:
- Ultraviolet treatments to your skin?
- Radiation treatments?
- Injuries to the skin?
- Skin infections?
Your doctor might also take blood tests and a skin biopsy, a sample from your skin, to send to a lab for examination.
Bullous Pemphigoid Treatment
Bullous pemphigoid treatment depends on how serious it is. If you have open blisters and they’re leaking fluid, this is serious. These are open wounds that can become infected. You should get medical care within 24 hours of noticing these to reduce the risk of getting a serious infection.
If you have a mild case of bullous pemphigoid and no open blisters or wounds, you might only need a topical corticosteroid ointment and/or emollient cream to apply directly to your skin. The corticosteroid would help relieve the itching and the emollient would help make your skin softer.
If your condition is moderate to severe, your doctor might recommend one or more of these treatments:
- Stronger topical corticosteroid creams or ointments
- Oral corticosteroids, like prednisone
- Antibiotics, like:
- Immunosuppressant medications like methotrexate
- Other drugs like:
- Dupilumab
- Omalizumab
- Rituximab
- Intravenous (IV) immunoglobulin
- Pain relievers
Blisters and open wounds must be taken care of to prevent infection. Ask your doctor how best to do this. Many products are on the market to use as dressings to protect the wounds, ranging from gauze that has petroleum jelly in it (to prevent sticking) to special foam dressings. The important thing is not to use a dry dressing that will stick to the wound or blister when it gets dry. It will become hard to remove and could cause more injury to the skin.
If the blisters are widespread and severe enough, you might need specialized wound care in a hospital burn unit, where the staff has special expertise in wound care.
It can take several years for bullous pemphigoid to go away completely. In the meantime, your doctor will monitor your progress to see how the treatment is working, and may switch drugs if needed. Some medications can have serious side effects, so report anything unusual to your doctor. You might need regular tests, like a bone density (DEXA) scan, blood pressure monitoring, or blood tests to make sure that you aren’t having any hidden side effects.
Managing Bullous Pemphigoid
If your doctor prescribes treatment to manage the blisters and other bullous pemphigoid symptoms, it can take a while before you see or feel any changes. In the meantime, there are some things that you can try at home to help your skin feel at least a bit better:
- Use mild antibacterial soap when washing. Don’t rub to dry. Instead, gently dab your skin.
- Apply antibiotic ointment or cream to the affected areas, especially where the skin has broken.
- Don’t allow anything to rub against your skin. Wear loose clothing, and it’s best if it’s cotton or some other natural fiber.
- Moisturize your skin well with lotions, ointments, or creams. Shea butter oil and coconut oil are also good products to use on your skin.
- If you have bullous pemphigoid on your feet, try not to stand or walk for too long.
Some people have bullous pemphigoid in their mouth, and that can make eating and drinking challenging. Here are some tips that might make it easier:
- Eat soft or liquid foods, like soup, applesauce, mashed potatoes, etc.
- Let hot food cool down a bit before eating.
- Avoid:
- Anything that is sharp or crunchy, such as chips, toast, or raw vegetables
- Acidic foods, like citrus fruit
- Spicy foods, like hot peppers and salsa
- Alcohol
Bullous Pemphigoid Complications
People with bullous pemphigoid can see their disease go away as quickly as a few months or as long as up to five years. During that time, there can be some complications.
- Bacterial infections, especially staphylococcal and streptococcal infections
- Viral infections, including herpes simplex and varicella zoster
- Sepsis, your body’s overall toxic response to an infection
- Side effects from treatments
When Should I See My Doctor?
It’s a good idea to check in with your doctor if you have any skin issues that are making you uncomfortable, but it’s especially important to go if you have:
- Blisters that you can’t explain
- Blisters on your eyes
- Any signs of infection, such as:
- Increasing pain
- Redness or warmth around a blister or wound
- Pus leaking from a broken blister or wound
- Fever
Takeaways
Bullous pemphigoid is an autoimmune disease that affects your skin. It’s rare, but the chances of getting it increase with age. People with some other autoimmune diseases, like multiple sclerosis and rheumatoid arthritis, as well as those who have dementia, have a higher risk of getting it. Bullous pemphigoid disease causes large blisters to form all over your body and it can take years of treatment for it to go away. Infections are a risk if you have bullous pemphigoid, so you must take care of your skin, treating it gently and following treatment instructions.
Bullous Pemphigoid FAQs
What is your life expectancy if you have bullous pemphigoid?
Bullous pemphigoid is not a fatal illness, so it doesn’t affect your life expectancy.
Is bullous pemphigoid contagious?
Bullous pemphigoid is an autoimmune disease. It is not contagious.
Does bullous pemphigoid ever go away?
Bullous pemphigoid does go away, but it can take as long as five years in some people.
What foods should you avoid with bullous pemphigoid?
Only people who have bullous pemphigoid in their mouth need to avoid certain foods because they can increase the pain. So this means avoiding anything that can cut or feel sharp (like chips or toast) or is too hot, acidic, or spicy.
Can bullous pemphigoid be caused by stress?
Stress doesn’t cause bullous pemphigoid, but it can cause it to flare up.
Which medications can trigger bullous pemphigoid?
Some prescription medications can trigger bullous pemphigoid. They include alogliptin, etanercept, furosemide, penicillin, and sulfasalazine.
What conditions can be mistaken for bullous pemphigoid?
There are some skin conditions that could mimic or be mistaken for bullous pemphigoid: atopic dermatitis, irritant contact dermatitis, dermatitis herpetiformis, urticaria, and even severe poison ivy.
