Pemphigoid

Pemphigoid is a rare blistering condition that mainly affects people over 60 years old. While it can be serious and last for years, proper treatment helps control the symptoms and allows the skin to heal, though it may take time and patience.

Table of contents

• What is pemphigoid?
• Who gets pemphigoid?
• What causes pemphigoid?
• What are the symptoms?
• How is pemphigoid diagnosed?
• How is pemphigoid treated?
• What complications can occur?
• What is the outlook?
• Self-care tips

What is pemphigoid?

Pemphigoid is a group of rare autoimmune blistering diseases that affect the skin and sometimes the mucous membranes, such as the inside of the mouth. An autoimmune disease occurs when your body’s immune system, which normally protects you from infections, mistakenly attacks healthy tissues^[1].

Bullous pemphigoid is the most common type of pemphigoid, representing about 80% of cases. It causes large, fluid-filled blisters on the skin^[3]. The blisters are typically tense and firm, meaning they don’t break easily. This is different from a similar condition called pemphigus, where the blisters are fragile and break open quickly^[8].

A less common form called mucous membrane pemphigoid primarily affects moist areas of the body, including the mouth, eyes, nose, throat, and genital areas^[3].

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Who gets pemphigoid?

Bullous pemphigoid most commonly affects people over 60 years old, with many cases occurring in those over 80. The condition can develop in younger adults, but this is less common. Bullous pemphigoid in infants and children is rare^[6].

The condition affects men and women equally. The annual rate of new cases is between 6 and 13 per million people in the United States^[4].

Certain groups of people appear to be at higher risk. The condition is more common in older patients with neurological diseases, particularly stroke, dementia, and Parkinson’s disease^[6]. People with psoriasis also have a greater risk of developing bullous pemphigoid, and it can be triggered by treatment of psoriasis with light therapy^[6].

What causes pemphigoid?

Pemphigoid is caused by a malfunction of the immune system. In this condition, your immune system produces proteins called autoantibodies that attack the basement membrane, which is the layer of tissue that connects the outer layer of skin to the deeper layers^[1].

Healthcare providers don’t fully understand why this immune system response happens. In some people, certain factors may trigger the condition^[5]:

• Certain medications
• Sunburn
• Surgery
• Burns
• Skin infections

Several types of medications have been linked to pemphigoid. The most common are checkpoint inhibitor immunotherapies, such as pembrolizumab and nivolumab, which are used to treat cancer. Pemphigoid can develop after 12 months of therapy or even after these medications are stopped^[6].

Diabetes medications called dipeptidyl peptidase-4 inhibitors (often called “gliptins”), such as sitagliptin and vildagliptin, have also been reported to cause bullous pemphigoid. The condition typically appears about 11 months after starting these medications^[6].

Other medications that may trigger pemphigoid include diuretics such as furosemide and spironolactone, certain antibiotics, and some blood pressure medications^[4].

Pemphigoid is not hereditary, meaning it cannot be passed on to your children. It is not caused by an infection or allergy, cannot be passed from person to person, and is not affected by diet or lifestyle^[5].

What are the symptoms?

Bullous pemphigoid usually starts with itchy skin, which can be severe. This itching may begin weeks or even months before any blisters appear^[1].

Early in the disease, you may develop an itchy, raised rash that can look like eczema or hives (nettle rash). The affected patches may appear red or pink on light skin and dark reddish-brown on darker skin tones^[2].

After a few weeks, blisters may develop. The main characteristics include^[1]:

• Large blisters that don’t break easily
• Blisters often appear along skin folds or creases, such as the upper thighs, armpits, elbows, hands, feet, and abdomen
• The fluid inside blisters may be clear, cloudy, yellowish, or contain blood
• The skin around blisters may look normal or may be discolored
• When blisters break, they leave sores that can be painful

Some people may have blisters or sores in the mouth, though this is rare. Roughly a quarter of patients with pemphigoid have blisters or sore areas inside the mouth^[7]. When blisters occur in the mouth, gums, or other moist areas, this may indicate mucous membrane pemphigoid^[5].

When the skin heals, it usually doesn’t leave scars, but there may be changes in skin color after the inflammation settles down^[7].

• Skin
• Mouth
• Eyes
• Throat
• Genital areas

How is pemphigoid diagnosed?

A healthcare professional will talk with you about your symptoms and medical history and conduct a physical examination. The appearance of the rash is often enough for a doctor to suspect pemphigoid, but further tests are needed to confirm the diagnosis^[5].

Your healthcare professional may refer you to a specialist in skin conditions, called a dermatologist^[10].

Diagnostic tests may include^[10]:

Skin biopsy: A biopsy is a procedure to remove a small sample of tissue for testing in a laboratory. This allows doctors to examine the skin under a microscope. Special techniques called direct immunofluorescence can show the presence of autoantibodies at the basement membrane zone^[4].

Blood tests: Blood tests can detect the presence of specific autoantibodies in your bloodstream. An ELISA test is useful for diagnosing bullous pemphigoid by detecting these antibodies^[4].

How is pemphigoid treated?

Treatment of bullous pemphigoid aims to heal the skin, relieve itching and pain, and prevent new blisters from forming. Your healthcare professional will likely prescribe one or a combination of medicines^[10].

Corticosteroids are the main treatment for bullous pemphigoid. These medications reduce inflammation and suppress the immune system^[10].

For localized disease affecting small areas, strong steroid creams such as clobetasol propionate may be prescribed. Steroid creams or ointments are much less likely to cause side effects than steroid tablets^[5].

For more extensive blistering, steroid tablets taken by mouth may be needed to get the pemphigoid under control. While these can be very effective, oral steroids carry risks of harmful side effects, such as weakened bones, diabetes, stomach ulcers, and eye problems^[10].

The treatment is usually taken for a few weeks or months. It typically takes about three weeks for new blisters to stop forming and about eight weeks for existing blisters to heal. Some patients can gradually come off their treatment after a few months, while others will need to continue with lower doses for several years^[5].

Antibiotics such as doxycycline and dapsone can help control blisters and reduce inflammation^[10].

Immunosuppressant medications may be used alongside steroids or to allow lower doses of steroids to be used. These medicines stop your immune system from attacking healthy tissues. Examples include azathioprine, mycophenolate mofetil, and methotrexate. These medications can have serious side effects, including increasing the risk of infection, so patients need close monitoring^[10].

Biologic therapies are newer treatments that target specific parts of the immune system. Rituximab, an antibody that targets certain immune cells, has shown promising results in patients with severe or difficult-to-treat pemphigoid^[14]. Dupilumab, which blocks certain immune signals, has demonstrated high disease control rates with rapid relief of itching and good safety^[15]. Omalizumab, which blocks a type of antibody called IgE, can also be effective^[15].

If you have blisters in your mouth, your doctor may prescribe a medicated mouthwash to help with pain and healing^[5].

You may also be given tablets to protect your bones and an anti-acid tablet to protect your stomach lining if you’re taking steroid tablets^[5].

What complications can occur?

Bullous pemphigoid can sometimes cause serious problems and may be life-threatening, especially for people at higher risk of complications^[2].

The main risks include^[2]:

Skin infections: When blisters break, they leave open sores that can become infected. These infections can be very serious if they spread deeper into the body, a condition called sepsis.

Side effects from treatment: Long-term use of steroid medications can cause high blood pressure, weakened bones, and a higher chance of getting infections. Healthcare providers try to use steroids at the lowest possible dose and for the shortest time necessary to help avoid these side effects.

It’s important to attend regular check-ups with your doctor so that any problems can be found and treated early^[2].

What is the outlook?

Pemphigoid cannot be cured completely, but different treatments can control the condition well. Treatment helps the skin to heal, stops new blisters from forming, and reduces the risk of complications^[5].

Bullous pemphigoid often goes away on its own eventually, though this may take between one and five years. Some people experience a long-term remission after treatment, while others may need ongoing treatment^[7].

Your skin should eventually heal without scarring, though it might be a bit darker than it was before^[2].

Self-care tips

There are several things you can do to help manage pemphigoid and make yourself more comfortable:

Managing medications: Do not change the dosage of steroid tablets you are taking without talking to your doctor first. Ask your doctor about what dose of steroids to take if you become unwell, as you may need adjusted doses during illness^[5].

Caring for blisters: A nurse can show you how to safely burst and dress blisters to help relieve pressure and discomfort. Once you’ve been shown the proper technique, you can do this yourself. It’s important not to burst your blisters yourself unless you have been shown how to do so safely and hygienically^[2].

Skin care: When bathing, avoid scrubbing your skin. Use a gentle cleanser and lightly pat dry with a towel, leaving the skin slightly damp. Avoid sun exposure and heat. Dress in soft, breathable clothing^[20].

Oral care: If you have sores in your mouth, avoid spicy, acidic, or salty foods, as these can irritate the sores. Use a soft toothbrush and gentle, mint-free toothpaste. Avoid harsh brushing of the gums. Use a non-alcohol-based antiseptic mouthwash. Maintain regular dental check-ups^[5][20].

Diet modifications: Avoid foods that are crunchy (nuts, chips, crisp raw vegetables), very hot (steaming foods or hot beverages), or acidic (tomatoes, citrus juices)^[20].

Managing stress: Stress can worsen autoimmune conditions. Regular physical exercise can help with stress management, though you should avoid contact sports. Consider practicing relaxation techniques such as meditation or deep breathing. Have someone you can talk openly with about your condition^[20].

Watch for signs of infection: Be alert for signs that your skin might be infected, such as increased pain, swelling, heat, or pus. Contact your healthcare provider promptly if you notice these signs^[2].

Learn about your condition: Understanding your illness, including its signs, symptoms, treatment, and management strategies, can help you take better care of yourself. Keep a journal to track your symptoms, questions for your healthcare team, and the courses of action being taken^[19].