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Bullous pemphigoid

Contributors: Taylor Williams, Chase W. Kwon MD

Overview

Bullous pemphigoid (BP) is a rare condition where large fluid-filled blisters develop on the skin. It is caused by an autoimmune reaction, where the body’s immune system mistakenly attacks proteins in the membrane beneath the skin. This leads to inflammation and blister formation. These blisters can be very itchy.

The symptoms of BP can vary. A person can have mild itching with no rash, or they could have widespread blistering. And some people have milder disease that improves over time, while others have disease that lasts months to years. While the disease can affect the skin on any part of the body, the blisters most often occur on the chest, back, and on the folds of the arms and legs. Sometimes the inside of the mouth can be affected. Although the blisters can become infected, BP itself is not contagious.

BP has been associated with other medical conditions, including stroke and dementia, as well as other autoimmune diseases, such as diabetes, thyroiditis, and rheumatoid arthritis. Certain medications, especially ones used to treat cancer, blood pressure, and diabetes, have also been associated with BP.

Who’s At Risk

BP typically affects older adults and usually starts around age 60. However, it can occur at any age, and it can affect anyone of any race and/or ethnicity or sex. When BP occurs in younger individuals, the medical professional may want to review all the medications they are taking, even over-the-counter medications such as ibuprofen and naproxen, in case a medication might be causing their BP.

Signs & Symptoms

BP can range from mild itching and no rash to a widespread blistering rash. Individuals with BP may have:

• Hives or eczema-like patches, especially in the early stage.
• Severe itching, sometimes bad enough to interfere with sleep.
• Small fluid-filled blisters.
• Large fluid-filled blisters.
  • Skin abrasions, or scratches.
• An increase in a type of white blood cell seen on bloodwork, called peripheral eosinophilia.

Self-Care Guidelines

In addition to medical treatment, there are steps you can take at home to manage your BP:

Keep your skin clean and dry.

Avoid scratching or rubbing the affected areas, as this can worsen the condition.

Wear loose-fitting, comfortable clothing made from soft fabrics to reduce friction on your skin.

·When caring for blisters and abrasions, gently cleansing and soaking the affected areas with lukewarm water mixed with Domeboro powder (aluminum sulfate and calcium acetate) can be helpful.

If uncomfortable, intact blisters may be carefully punctured with a sterile needle andallowed to drain, but the blister roof should be left intact to serve as a natural bandage.

·Protect your skin from excessive sun exposure by using a broad-spectrum sunscreen (that protects against both UVA and UVB rays) of SPF 30 or higher and wearing sun-protective clothing.

When to Seek Medical Care

If you suspect you may have BP, see a medical professional such as a dermatologist for diagnosis and treatment. Taking photos of your blisters and rash and sharing them with your medical professional can help because the blisters may be gone by the time of the appointment.

Your medical professional may perform a skin biopsy and order bloodwork to confirm the diagnosis.

If you have been diagnosed with BP, regular follow-up visits with your medical professional areessential to monitor your condition, adjust treatment as needed, and address any concerns or changes in symptoms.

Treatments

Treatment for BP aims to reduce inflammation, control itching, and prevent blister formation. Treatment options may include:

• Corticosteroid creams or ointments for milder cases.
• Doxycycline or minocycline (antibiotics often used for their anti-inflammatory properties to treat skin conditions like acne and rosacea) in combination with nicotinamide (vitamin B3) for milder cases.
• Oral corticosteroids for more severe cases.
• Immunosuppressive medications to lessen the immune system’s response.
• Dupilumab (Duplixent) as an “off-label” use (as this medication is currently US Food and Drug Administration [FDA] approved for the treatment of eczema and is not indicated for the treatment of BP).
• Antibiotics, if affected areas of skin become infected.

If a medication is potentially causing your BP, your medical professional will work with you to determine if stopping the medication is safe.

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