Pemphigus

What is pemphigus?

Pemphigus is a group of autoimmune skin conditions that cause sores, blisters or fluid-filled bumps to form on your skin. These blisters can also form in your mucous membranes, which are the soft linings of your eyes, nose, mouth, throat and genitals.

The blisters are soft and break open easily to form painful sores. Without treatment, they can spread over large areas of your body and have a risk of infection.

Pemphigus is sometimes confused with other autoimmune blistering skin conditions such as bullous pemphigoid, lupus erythematosus and Hailey-Hailey disease.

Pemphigus isn’t contagious. It’s a lifelong condition that can be managed with ongoing medical treatment.

What are the types of pemphigus?

There are several types of pemphigus based on where and why lesions develop. Types of pemphigus include:

• Pemphigus vulgaris: This is the most common type in the U.S. Blisters always affect your mouth. Some people may have blisters on their skin and in other mucous membranes. These lesions develop in superficial layers of your skin. They can be painful and heal slowly.

Pemphigus vulgaris causes red and white fluid-filled blisters or open sores to form inside of your mouth.

Pemphigus vulgaris causes blisters to form on your skin. A common location for blisters is near your groin and on the skin on your legs.

• Pemphigus vegetans: This type is similar to pemphigus vulgaris but causes thicker lesions. These lesions usually form in areas of your body with skin folds such as your groin and armpit.
• Drug-induced pemphigus: Medications can cause blistering. Some drugs that cause this condition include antibiotics and blood pressure medication. Blisters can develop months after taking the medicine.
• Pemphigus erythematosus (Senear-Usher syndrome): This type is an overlap syndrome with lupus that causes blisters to develop on your upper back, chest, cheeks and scalp. When lesions form, they’re usually red and scaly.
• Pemphigus foliaceus: This type causes blisters to develop on your scalp, face, neck and back. Lesions rarely appear in your mouth. This type affects your outermost skin layer only. Small blisters may break open easily to form crusty lesions that spread to cover large areas of skin.

Pemphigus foliaceus causes red to purple blisters to form most often on your back in groups that affect the outside layer of your skin. These blisters can easily spread to cover a large area of your skin.

• Endemic pemphigus (fogo selvagem): This is a form of pemphigus foliaceus that occurs more often in South and Central America, particularly Brazil.
• Paraneoplastic pemphigus: This is the rarest type of pemphigus that develops in people diagnosed with cancer. Severe blisters form in your mouth. If your healthcare provider diagnoses paraneoplastic pemphigus, they’ll look for signs of cancer somewhere in your body.

Who does pemphigus affect?

Pemphigus can affect anyone. It’s most common among people between the ages of 40 and 60.

Specific geographical regions of the world have a higher number of cases, including:

• Southeast Europe.
• India.
• The Middle East.
• Tunisia.
• Brazil.

How common is pemphigus?

Pemphigus isn’t common. An estimated 1 to 5 out of every 1 million people receive a pemphigus diagnosis throughout the world each year.

How does pemphigus affect my body?

Pemphigus causes blisters and sores to form on your skin. These lesions form quickly and can last for years, with new blisters appearing in the same area of your skin after one blister goes away. These lesions can be painful and cause additional symptoms like infections. Contact your healthcare provider if you have blisters that are widespread across your body, as they could be life-threatening.

What are the symptoms of pemphigus?

Symptoms vary based on the type of pemphigus you have but could include:

• Fluid-filled bump or bubble on your skin (blister).
• The skin around the blister is pink to red.
• Sores that have a crusty appearance.
• Blisters or sores leak clear fluid or bleed lightly.
• Your skin around the blisters is fragile and peels in layers or scales.
• Pain on or near your affected skin.
• Itchy skin.

Blisters and sores can easily become infected. Skin symptoms of an infection include:

• White or yellow pus fills the blister and leaks out if the blister breaks open.
• Pain or a burning sensation to the touch.
• Yellow crust forms on the blister if it breaks open.
• Skin doesn’t heal.
• The area on or around the blister swells or gets bigger.

Severe symptoms of pemphigus include:

• Fever.
• Fatigue.
• Muscle weakness.
• Vision problems and light sensitivity.

Where does pemphigus form on my body?

Pemphigus can form on your skin in different parts of your body. The most common places include your:

• Mouth and throat.
• Genitals.
• Face (cheeks, nose, eyes).
• Scalp.
• Back.
• Armpit.
• Chest.

What causes pemphigus?

The exact cause of pemphigus is unknown. Research suggests that genetics and environmental factors play a role in your diagnosis.

Pemphigus is an autoimmune condition. This means that your body’s defense system (immune system antibodies) attacks your body’s healthy cells, mistaking them for foreign invaders. When your body attacks itself, you’ll notice symptoms of pemphigus in the form of blisters or sores on your skin.

In rare cases, certain medications, including penicillin, an antibiotic, piroxicam, a nonsteroidal anti-inflammatory drug used for rheumatoid arthritis, and blood pressure medications can cause the condition.

Some studies found that specific HLA genes, which are genes that build your immune system, predispose you to certain types of the condition.

Is pemphigus contagious?

No, pemphigus isn’t contagious. You can’t spread the condition to other people.

How is pemphigus diagnosed?

Your healthcare provider will diagnose pemphigus after performing a physical exam, learning more about your medical history and offering tests that include:

• Biopsy: They’ll take a small sample of your skin’s tissue and examine it under a microscope.
• Blood tests: They’ll examine a sample of your blood to look for antibodies that cause the condition.

How is pemphigus treated?

Treatment is unique to each person diagnosed with pemphigus and could include:

• Taking medicine to prevent infections and help your skin heal.
• Stopping the use of medicines that cause your symptoms.
• Wound care for blisters and sores.

Your healthcare provider will treat your condition in stages. Most people go through all three stages of treatment, which include:

• Control: High doses of medications control the spread of blisters and begin healing existing ones.
• Consolidation: Steady doses of medications continue healing blisters until most clear up.
• Maintenance: Reduced levels of medications keep new blisters from forming.

What medicines treat pemphigus?

Medicines used to treat pemphigus include:

• Corticosteroids: Medication to reduce inflammation (swelling), delivered by mouth, by injection (a shot) or topically (ointments or creams).
• Immunosuppressive drugs: Drugs that manage your body’s autoimmune response, which is what happens when your body attacks healthy cells.
• Rituximab: A monoclonal antibody that targets problematic B cells.
• Intravenous immunoglobulin: Healthy antibodies (proteins made by your immune system to attack foreign substances), given through a needle into your vein, to help reduce the antibodies that cause your diagnosis.
• Antibiotics: In some cases, infections can develop in pemphigus blisters. If this happens, your doctor may prescribe antibiotic medicine to treat the infection.

Are there side effects of the treatment?

Side effects vary based on the type of treatment and could include:

• Chest pain.
• Dizziness.
• Headache.
• Joint pain.
• Nausea.
• Upset stomach.
• Weight gain.

Your healthcare provider will monitor your condition regularly and order blood and/or urine tests to verify that your treatment is working. Tests also check for negative reactions to medications that treat pemphigus.

What can I eat or drink with pemphigus?

Eating and drinking can be difficult if you develop blisters in your mouth and throat. Choose foods that are soft and bland. Avoid foods that are crunchy, acidic and spicy, which could irritate your blisters and cause pain.

If you have trouble eating, contact your healthcare provider. They may recommend taking nutritional supplements to avoid malnutrition.

How do I take care of myself and manage my symptoms?

You can take steps at home to manage your symptoms of pemphigus by:

• Avoiding crunchy, acidic or spicy foods that can irritate mouth and throat blisters.
• Caring for your blisters as directed by your healthcare provider.
• Staying out of the sunlight or covering your skin with sunscreen or wearing protective clothing to prevent UV ray damage to your skin.
• Using soaps and lotions designed for sensitive skin, without fragrances to prevent skin irritation.

How soon after treatment will I feel better?

Treatment for pemphigus takes time before you see results. With treatment, you’ll notice new blisters stop forming after several weeks and your skin will begin to heal. It could take months for your blisters and sores to heal completely.

How can I prevent pemphigus?

As the cause of pemphigus is unknown, there isn’t a way to prevent the condition.

What can I expect if I have pemphigus?

The majority of people diagnosed with pemphigus have a normal lifespan. Treatment helps manage symptoms and could be ongoing throughout your life.

If left untreated, symptoms can affect your overall health and could cause life-threatening symptoms.

Is there a cure for pemphigus?

No, there isn’t a cure for pemphigus. Treatment is effective to alleviate symptoms.

When should I see my healthcare provider?

Contact your healthcare provider if you:

• Develop blisters on your skin or in your mouth that heal very slowly or don’t heal at all.
• Develop blisters on a large area of your body.
• Experience severe pain or discomfort.
• Have an infection, when your blisters break and leak a white to yellow fluid or your skin around your blisters get bigger (swell).

What questions should I ask my doctor?

If you have pemphigus, you may want to ask your healthcare provider:

• How serious is my diagnosis?
• What are my treatment options?
• What signs of complications should I look out for?

What’s the difference among pemphigus, bullous pemphigoid and lupus?

While pemphigus, bullous pemphigoid and lupus are skin conditions, there are differences among the three conditions.

Pemphigus is an umbrella term for a group of skin conditions that cause blister-like lesions to form on your skin and mucus membranes.

Bullous pemphigoid is another rare skin condition that causes itchy, hive-like welts and fluid-filled blisters on your skin. Bullous pemphigoid most often affects people older than 65 years and can be life-threatening.

Lupus is an autoimmune condition that causes pain and swelling throughout your body. It can cause skin rashes to form because your body’s healthy cells attack your skin’s tissues.

What resources are available for people with pemphigus?

There are several resources to help support people with pemphigus. Many people with the condition join support groups to share experiences and ways to manage their diagnosis.

The International Pemphigus & Pemphigoid Foundation and RareConnect are two resources that provide education and support to people with pemphigus and their caregivers.

A note from Cleveland Clinic

Pemphigus is a group of skin conditions that can be persistent and ongoing throughout your life. Your healthcare provider will work closely with you to help you manage your symptoms and eliminate discomfort, especially to prevent infections. Be patient with your body to heal and try not to injure your blisters or skin sores. If you notice blisters form on your skin without a cause, contact your healthcare provider.