Bullous pemphigoid

Bullous pemphigoid (BUL-us PEM-fih-goid) is a rare skin condition that causes large fluid-filled blisters. They often appear on the skin near creases, such as the upper thighs and armpits. Sometimes, people get a rash instead of blisters. The affected areas may be painful and are usually very itchy. Blisters or sores also might form in the mouth, but this is rare.

Bullous pemphigoid occurs when the immune system attacks a layer of tissue in the skin. The reason for this immune system response is not well understood. In some people, the condition is brought on by certain medicines.

Bullous pemphigoid often goes away on its own in a few months, but it may take as many as five years to go away for good. Treatment usually helps heal the blisters and prevent new ones from forming.

The condition is most common in people over age 60.

The symptoms of bullous pemphigoid may include:

• Itching, which can start weeks or months before blisters form.
• Large blisters that don't break easily, often found along skin folds. On brown and Black skin the blisters may be dark pink, brown or black. On white skin they may be yellow, pink or red.
• Pain.
• A rash.
• Small blisters or sores in the mouth or other mucous membranes. This is a symptom of a rare type of the disease called mucous membrane pemphigoid.

When to see a doctor

See a healthcare professional if you have:

• Unexplained blisters.
• Blisters on your eyes.
• An infection.
• Blisters that are opening up and leaking.

The symptoms of bullous pemphigoid occur when the immune system attacks a layer of tissue in the skin. The cause of this problem is not well understood. In some cases, the condition is brought on by:

• Medicines. Several medicines are known to increase the risk of bullous pemphigoid. Examples are diuretics such as furosemide; antibiotics such as amoxicillin, penicillin and ciprofloxacin; NSAIDs such as aspirin and ibuprofen; diabetic medicines such as sitagliptin (Januvia); and medicines to treat cancer such as nivolumab and pembrolizumab.
• Light and radiation treatments. Ultraviolet light therapy to treat certain skin conditions may bring on bullous pemphigoid. Also, radiation to treat cancer can cause the condition.
• Medical conditions. Psoriasis, lichen planus, dementia, Parkinson's disease, stroke and multiple sclerosis are among the conditions that may be related with bullous pemphigoid.

The condition isn't an infection and it isn't contagious.

Bullous pemphigoid is most common in people over age 60, and the risk increases with age. The condition can be life-threatening for older people who have other conditions at the same time.

Possible complications of bullous pemphigoid include:

• Infection.
• Changes in skin color after the affected skin heals. This change in skin color is called post-inflammatory hyperpigmentation when the skin darkens and post-inflammatory hypopigmentation when the skin loses color. People with brown or Black skin have a higher risk of long-term skin color changes.
• Side effects from the medicine used to treat bullous pemphigoid.

Your healthcare professional will talk with you about your symptoms and medical history and conduct a physical exam. You may need tests to confirm a diagnosis of bullous pemphigoid. These may include blood tests, a skin biopsy or both. A biopsy is a procedure to remove a sample of tissue for testing in a lab.

Your healthcare professional may refer you to a specialist in skin conditions. This type of doctor is called a dermatologist.

Treatment of bullous pemphigoid is aimed at healing the skin, relieving itching and pain, and preventing new blisters. Your healthcare professional will likely prescribe one or a combination of medicines:

• Corticosteroids. The main treatment for bullous pemphigoid is corticosteroid medicine applied to the affected area. Usually a strong steroid cream such as clobetasol propionate is used. Long-term use this type of medicine comes with the risk of skin thinning and easy bruising. Your healthcare professional might also suggest a steroid medicine taken by mouth. Oral steroids come with the risk of harmful side effects, such as weak bones, diabetes, stomach ulcers and eye problems.

Depending on how you respond to the first medicines you try, your healthcare professional may suggest something other than steroids.

• Antibiotics. The oral medicines dapsone and doxycycline help control blisters.
• Medicines that target the immune system. Some medicines can stop your immune system from attacking healthy tissues. Examples are azathioprine (Azasan, Imuran), rituximab (Rituxan), mycophenolate (CellCept) and methotrexate (Trexall). These medicines also have a risk of harmful side effects, including infection. People who take these medicines need close follow-up and, at times, repeat blood tests to monitor for side effects.

Bullous pemphigoid is usually goes away in time. Sores can take weeks to heal, and it's common for new ones to form.

If you have bullous pemphigoid, you can help take care of your condition with the following self-care strategies:

• Following wound care advice. Follow your healthcare professional's advice for care of blisters.
• Limiting activities if needed. Blisters on the feet and hands can make it difficult to walk or to go about daily tasks. The itching may make it hard to sleep. You may need to change your routine until the blisters are under control.
• Protecting your skin. Try not to scratch the affected area. And protect your skin from too much heat and sun, even on cool, cloudy or hazy days.
• Wearing loose-fitting cotton clothes. This helps protect your skin.
• Watching what you eat. Don't eat hard and crunchy foods if you have blisters in your mouth.

Bullous pemphigoid may be difficult to live with, especially if it affects your daily activities or causes lost sleep or stress. You may find it helpful to talk with others who have or had bullous pemphigoid. You may want to connect with a support group in person or online. Ask your healthcare professional for suggestions.

You're likely to first see your regular healthcare professional. You may then be referred to a healthcare professional who specializes in skin disorders. This type of doctor is called a dermatologist.

Here's some information to help you get ready for your appointment.

What you can do

Before your appointment make a list of:

• Symptoms you've been having and for how long.
• The name and contact information of any healthcare professional you have seen recently.
• All medicines, vitamins and supplements you take, including doses.
• Questions to ask your healthcare professional.

For bullous pemphigoid, some basic questions to ask your healthcare professional include:

• What's the most likely cause of my symptoms?
• Do I need any tests?
• How long will it take for the blisters to heal? Will they leave scars?
• Will the blisters come back again?
• What can I do for the pain?
• How long will these skin changes last?
• What treatments are available, and which do you recommend?
• What side effects can I expect from treatment?
• I have other health conditions. How can I best manage them together?
• Is there a generic alternative to the medicine you're prescribing me?
• Do you have any brochures or other printed material I can take with me? What websites do you recommend?

What to expect from your doctor

Your healthcare professional is likely ask you a number of questions, such as:

• When did these symptoms begin?
• Where are the blisters located? Do they itch, ooze, drain pus or bleed?
• Have you started any new medicines?
• Have you had a fever?
• Does anything seem to improve your symptoms?
• What steps have you taken to treat this condition yourself?
• Have any of these measures helped?
• Have you ever been treated by a healthcare professional for this condition?
• If so, did you use any prescription treatments for this skin condition? If so, do you remember the name of the medicine and the dosage you were prescribed?
• Did you have a skin biopsy?