What is Bullous Pemphigoid?

Bullous pemphigoid (BUL-us PEM-fih-goid) is a rare skin condition. It causes large fluid-filled blisters to form on your skin. They often start on areas of your skin that flex (bend). These areas include your arms, armpits, legs, lower abdomen (belly), and groin (the area between your belly and thigh).

Who Gets Bullous Pemphigoid?

Bullous pemphigoid is most common in older females, age 70 and older. It’s not as common in males, younger adults, and children.

Some people who are pregnant can get bullous pemphigoid. This is called gestational pemphigoid. Your dermatologist (skin doctor) and obstetrician (doctor with special training in pregnancy and childbirth) will work together with you to help you manage it.

Bullous pemphigoid is not contagious (cannot spread from person to person). This means you can’t catch it from someone who has it.

Symptoms of Bullous Pemphigoid

The most common symptom of bullous pemphigoid is small patches of itchy skin. This is usually the first symptom.

• You may also get a pink-colored rash (see Figure 1) that looks like eczema (skin rash).
• Some people may get blisters after a week or more (see Figure 2), while others may get blisters months later. These blisters are often itchy, but not painful. They’re mostly firm and dome shaped.
• The fluid inside the blisters is usually clear but can also be cloudy or have a little blood in it. The skin around your blisters may look normal, darker than normal, or red.

In very rare cases it has been reported that people don’t witness any symptoms of Bullous Pemphigoid but those who do they report the following symptoms:

Causes of Bullous Pemphigoid

We don’t know what causes bullous pemphigoid. But we do know that it starts when your body’s immune system (your body’s defense against infection) makes antibodies (proteins) that attack healthy cells. This is called an autoimmune response.

Your antibodies attack the membrane between the outer layer of your skin (epidermis) and the layer under it (dermis). This attack causes fluid to build up and form between these 2 layers of your skin.

Certain things have been linked to bullous pemphigoid, but we don’t think they cause it. They include:

Diseases, such as:

• Ulcerative colitis (sores in your colon).
• Multiple sclerosis (a disease that affects your central nervous system).

Medications, such as:

• Furosemide (reduces extra fluid in your body).
• Non-steroidal anti-inflammatory drugs (NSAIDs).
• Captopril (treats high blood pressure).
• Penicillamine (treats rheumatoid arthritis).
• Some antibiotics (drugs that kill bacteria).
• Ultraviolet radiation and radiotherapy

Where do Bullous Pemphigoid Blisters Appear on your Skin?

Any area of your skin can be affected, but blisters often appear on your:

• Arms
• Armpits
• Legs
• Lower abdomen (belly)

Groin (the area between your belly and thigh).
There may be more blisters on certain parts of your body than others, such as your lower leg. In severe (very bad) cases, your whole body may be affected.

Some people may get small blisters inside their mouth. These blisters can open and look like mouth ulcers.

How is Bullous Pemphigoid Diagnosed?

If your healthcare provider suspects that you have bullous pemphigoid, they will send you to a dermatologist. These tests are done to confirm your diagnosis:

• A biopsy. This is a procedure to take a tissue sample. The sample is looked at under a microscope and tested to confirm bullous pemphigoid caused the blisters.
• A blood test. This can find the antibody that causes bullous pemphigoid. The antibody can also be found in your urine (pee) or blister fluid.

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

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.

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.

Reference:

https://my.clevelandclinic.org/health/diseases/15855-bullous-pemphigoid

https://www.mskcc.org/cancer-care/patient-education/bullous-pemphigoid-what-it-and-how-treat-it