Bullous pemphigoid: Diagnosis and treatment

How do dermatologists diagnose bullous pemphigoid?

Bullous pemphigoid is a disease that can cause widespread blisters, itching, and rash on your skin, and sometimes even inside your mouth and other areas.

If you are worried that you have bullous pemphigoid, seeing a board-certified dermatologist can help you get an accurate diagnosis and treatment.

Different types of diseases can cause blisters, and specialized testing may be necessary to find out what type of blistering disease you have. This specialized testing is usually performed by dermatologists.

To find out if you have bullous pemphigoid, your dermatologist will:

• Ask questions

• Examine your blisters and rashes

• Perform one or more skin biopsies (can be done during an office visit)

• Order blood tests, as needed

During your first appointment, your dermatologist will look for signs of infection as well as examine any blisters and rashes. If you have a skin infection or blisters cover a large area of your body, your dermatologist may admit you to a hospital. Having widespread blisters can lead to a type of infection called sepsis, which is a life-threatening condition.

If you have blisters that are leaking fluid and opening up, get immediate (within 24 hours) medical care

Treatment can prevent a serious — and sometimes life-threatening — infection.

Patients with widespread blisters may be admitted to a hospital’s burn unit. A burn unit provides the right environment, temperature, and humidity, along with the expertise needed to care for widespread blisters.

You may be admitted to the hospital before you are diagnosed with bullous pemphigoid. It takes time to get the results from medical tests.

If the medical tests confirm that you have bullous pemphigoid, your dermatologist will create a personalized treatment plan.

Have you had cancer, surgery, or radiation therapy?

Be sure to tell your dermatologist, as some treatments, surgeries, and medications can trigger bullous pemphigoid.

Will bullous pemphigoid go away without treatment?

This disease tends to come and go. You will likely have times when you continue to get new blisters and weeks when you have few — if any — blisters. Sometimes, this disease goes away without treatment and the person never develops blisters again.

Medical records show that before treatment was available for bullous pemphigoid, about 20% of patients went into remission after 15 months. This means that the itch, rash, and blisters went away after 15 months without treatment.

Remission differs from a cure. When a patient is in remission, the disease can come back. If the disease comes back, the patient is said to have a relapse.

If you have bullous pemphigoid, treatment can reduce the amount of time that it takes for bullous pemphigoid to go into remission.

For a few patients, remission begins 9 weeks after starting treatment. About 50% of patients go into remission about two years after treatment starts.

To stay in remission, you may need to continue treating bullous pemphigoid. Some patients continue to treat it for months to years to keep the disease under control.

How do dermatologists treat bullous pemphigoid?

Your dermatologist will tailor your treatment plan to your needs. The goals of treatment are to:

• Stop (or reduce) new blisters

• Heal existing blisters and sores

• Treat an infection if it has developed

• Relieve any itch and pain

A personalized treatment plan usually includes medication and wound care.

Treatment can help heal your skin so that bullous pemphigoid goes into remission

Treatment can also help relieve the itch and pain.

Medication: Your dermatologist may prescribe creams and ointments called corticosteroids. These help to heal your skin, prevent new blisters from appearing, and relieve the itch. If you have severe bullous pemphigoid, your dermatologist may prescribe corticosteroid pills.

Corticosteroids are powerful medications that act quickly to get your disease under control. Like other medications, corticosteroids can have side effects. Possible serious side effects include stomach ulcers or bleeding, bone loss that can lead to weak and fragile bones, eye problems, and diabetes.

To reduce your risk of developing side effects, your dermatologist may prescribe more than one medication. Adding a second or third medication may allow you to take a lower dosage of corticosteroids, which can reduce your risk of developing side effects.

If you do develop side effects, your dermatologist may team up with your primary care doctor to treat you.

Tell your dermatologist about all medications, vitamins, and supplements that you take — or plan to take

Some can interact with the medication in your treatment plan.

Other medications are also prescribed to treat bullous pemphigoid.

Tetracycline, doxycycline, and dapsone are antibiotics, which can reduce the inflammation inside your body. For some patients, applying a corticosteroid cream or ointment and taking an antibiotic provides effective treatment.

Some patients take a medication called an immunosuppressant. This medication helps to calm the immune system. If you need an immunosuppressant, your dermatologist may prescribe mycophenolate mofetil, methotrexate, azathioprine, or another medication that works on the immune system.

Before taking an immunosuppressant, make sure your vaccinations are up to date. Taking this type of medication can increase your risk of developing certain infections.

Dupilumab, omalizumab, or rituximab may be an option when other treatments fail to work. Instead of suppressing your immune system, these medications change the way your immune system reacts.

Wound care: Open blisters and raw skin can become infected. Wound care can prevent infection, relieve pain, and speed up healing.

Your dermatologist will create a wound care plan that meets your individual needs. Most wound care involves daily cleaning of the wounds, applying medication, and bandaging. When necessary, your dermatologist may refer you to a wound care specialist.

Treatment can take time

The amount of time you need to treat bullous pemphigoid will depend on how severe the disease is and your response to treatment.

Most patients follow a treatment plan for six months to five years before the disease goes into long-term remission. Once the disease is in long-term remission, many patients can stop treatment. However, some patients need to continue treatment.

Follow-up medical appointments are essential

Treating bullous pemphigoid can be complicated. Your dermatologist may coordinate with your primary care doctor and other doctors as needed. Keeping all of your medical appointments will help:

• Determine whether the treatment works for you or needs to be modified.

• Find side effects early.

• See if an infection, which can be serious, has developed.

• Make sure you know what to do, so you can follow your treatment plan.

Following a self-care plan for bullous pemphigoid can improve the results you get from treatment and how well you feel. You’ll find the self-care that dermatologists recommend at: Bullous pemphigoid: Self-care.

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References
Abdat R, Waldman RA, et al. “Dupilumab as a novel therapy for bullous pemphigoid: A multicenter case series.” J Am Acad Dermatol. 2020;83(1):46-52.

Bernard P, Borradori L. “Pemphigoid group.” In: Bolognia JL, et al. Dermatology. (fourth edition). Mosby Elsevier, China, 2018: 510-9.

Chan, LS. “Bullous pemphigoid.” In: Medscape (Elston DM., Ed.) Last updated 10/14/2020. Last accessed 7/9/2021.

Culton DA, Zhi L, Diaz LA. “Bullous pemphigoid.” In: Kang S, et al. Fitzpatrick’s Dermatology. (ninth edition) McGraw Hill Education, United States of America, 2019:944-55.

Mutasim DF. “Management of autoimmune bullous diseases: pharmacology and therapeutics.” J Am Acad Dermatol. 2004;51(6):859-77.

Grantham HJ, Stocken DD, et. al. “Doxycycline: a first-line treatment for bullous pemphigoid?” Lancet. 2017 Apr 22;389(10079):1586-8.

Han A. “A practical approach to treating autoimmune bullous disorders with systemic medications.” J Clin Aesthet Dermatol. 2009 May;2(5):19-28.

Nadelmann E, Czernik A. “Wound care in immunobullous disease.” IntechOpen. Published 5/9/2018. 10.5772/intechopen.71937. Last accessed 7/9/2021.

Yosipovitch G and Kwatra SG. “Itch associated with autoimmune disorders.” In: Living with itch: A patient’s guide. The Johns Hopkins University Press. United States of America, 2013: 63-5.

Written by:
Paula Ludmann, MS

Reviewed by:
Arturo R. Dominguez MD, FAAD
Ivy Lee, MD, FAAD
Shari Lipner, MD, PhD, FAAD

Last updated: 9/21/21