Bullous pemphigoid: Causes

What causes bullous pemphigoid?

Bullous pemphigoid is an autoimmune disease. This type of disease develops when your immune system malfunctions, causing your body to attack a part of itself.

When you have bullous pemphigoid, your body attacks fibers that connect the top layer of skin (epidermis) to the deeper layer (dermis).

This attack produces inflammation, which is what causes the skin to itch and the blisters to develop. Why the body starts attacking these fibers isn’t entirely clear.

Who has a higher risk of developing bullous pemphigoid?

Scientists have learned that some people have a higher risk of developing bullous pemphigoid.

Your risk increases if you:

• Are over 60 years of age

• Had a stroke

• Have dementia, epilepsy, multiple sclerosis, or Parkinson’s disease

It’s unlikely that your sex or race increase your risk. Bullous pemphigoid occurs equally in men and women. This disease also occurs in people of all races.

Bullous pemphigus usually develops after 60 years of age

Most people are in their 70s when they get this disease.

Bullous pemphigoid may have triggers

A trigger is something that either:

• Brings about a disease in someone whose genetic makeup increases the likelihood of developing that disease

• Causes symptoms in a person who already has a disease

There have been reports that the following can trigger bullous pemphigoid:

Certain prescription medications: While some medications have been reported to trigger this disease, it’s important to keep in mind that bullous pemphigoid is a rare disease. Many people who take one of the following medications never get bullous pemphigoid:

• Pembrolizumab, nivolumab: Given to treat melanoma and other cancers that have spread

• Saxagliptin, sitagliptin, vildagliptin: Diabetes medications

• Penicillin: An antibiotic

• Sulfasalazine: An anti-inflammatory

• Etanercept: A biologic

• Penicillamine: Given to treat Wilson’s disease or severe rheumatoid arthritis

Medical treatments: A few people have developed bullous pemphigoid after undergoing:

• Radiation therapy to treat cancer

• Ultraviolet light therapy

Surgery: There have also been reports of people developing bullous pemphigoid after having a:

• Colostomy

• Urostomy

• Skin graft

If a medical treatment has caused blisters, see a board-certified dermatologist. Treating bullous pemphigoid can prevent (or reduce) the new blisters that appear, reducing your risk of developing a serious infection.

To find out how dermatologists diagnose and treat this rare disease, go to: Bullous pemphigoid: Treatment.

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

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.

Heymann WR, “Untangling the neurologic comorbidities of bullous pemphigoid.” Dermatol World Insights & Inquiries. Last updated June 6, 2017. Last accessed July 9, 2021.

Tull TJ, Benton E. “Immunobullous disease.” Clin Med (Lond). 2021;21(3):162-5.

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