Pemphigus Foliaceus

What is pemphigus foliaceus?

Pemphigus foliaceus is a rare autoimmune condition that causes painful and itchy blisters and sores to form on your skin. The most common places for blisters and sores to form on your body are on your scalp, face, neck and back.

Blisters from pemphigus foliaceus form because the cells in your immune system produce proteins that attack your body’s healthy skin cells, mistaking them for foreign invaders.

Pemphigus foliaceus is a type of pemphigus, which is a group of skin conditions that cause blisters.

Who does pemphigus foliaceus affect?

Symptoms of pemphigus foliaceus arise on average between ages 50 and 60. The condition can affect any age, including children.

In certain parts of the world, specifically in South America, a form of pemphigus foliaceus, called fogo selvagem, may be related to an insect that is endemic in this area or another environmental trigger.

How common is pemphigus foliaceus?

Pemphigus foliaceus is a rare form of pemphigus. Pemphigus affects 1 to 5 out of every 1 million people throughout the world annually.

How does pemphigus foliaceus affect my body?

Pemphigus foliaceus causes painful blisters to form on your skin. The blisters are fragile and break open easily, which makes them turn into sores. When the blisters break, a scaly, crusty exterior forms on the affected area of your skin. These can be itchy and cause discomfort.

What are the symptoms of pemphigus foliaceus?

Symptoms of pemphigus foliaceus can range in severity and include:

• Blisters or small, fluid-filled, raised, red bumps on the top layer of your skin (epidermis) that can cover a large area of your body.
• Blisters are soft and break open easily.
• Broken blisters turn into sores that have a scaly, crusty texture.
• Blisters and sores cause itchiness, pain or a burning sensation.

Where do symptoms of pemphigus foliaceus affect my body?

The most common places on your body where you’ll find blisters and sores from a pemphigus foliaceus diagnosis are your:

• Scalp.
• Face.
• Neck.
• Back.
• Chest.

Symptoms don’t affect your mucous membranes, which are the linings to cavities in your body.

What causes pemphigus foliaceus?

There could be several possible causes of pemphigus foliaceus including both genetic and environmental factors. Studies are ongoing to learn more about the causes of this condition.

While the condition isn’t hereditary, meaning it can’t be passed to you from your parents during conception, you could be at an increased risk if you have a specific human leukocyte antigen (HLA) gene. HLA genes are responsible for identifying foreign invader proteins, like bacteria, to help your immune system destroy them to keep you healthy.

Pemphigus foliaceus is an autoimmune condition, which means that your immune system attacks healthy cells because it mistakes them for foreign invader cells. Specific to pemphigus foliaceus, the proteins that your immune system creates (antibodies) targets your cell’s adhesion points as invaders. Adhesion points are pins that hold the top layer of your skin cells (epidermis) together. When your body attacks these adhesion points, you experience symptoms of pemphigus foliaceus.

What causes a pemphigus foliaceus flare?

Symptoms of pemphigus foliaceus could flare if they’re triggered by environmental factors that include:

• Medicine: Certain non-steroidal anti-inflammatory drugs, penicillamine, nifedipine, captopril and enalapril can cause symptoms to flare or trigger disease. If your provider suspects these medicines cause your symptoms, they'll stop or change the medicine you take.
• Insect bite: In some regions of the world, specifically in South America, an insect bite may lead to endemic pemphigus foliaceus or fogo selvagem.
• Sun exposure: Exposing your skin to the sun’s UV rays can cause a flare of symptoms.

Is pemphigus foliaceus contagious?

No, pemphigus foliaceus isn’t contagious. You can’t spread the condition onto others by physical contact.

How is pemphigus foliaceus diagnosed?

Your provider will diagnose pemphigus foliaceus after learning more about your medical history and your symptoms. They will also physically examine the blisters or sores on your skin to verify that your symptoms match those of pemphigus foliaceus. Additional tests confirm a diagnosis.

What tests diagnose pemphigus foliaceus?

Your provider will confirm your diagnosis by offering a test, which could include:

• Skin biopsy: Your provider will take a small sample of your skin’s tissue and examine it under a microscope. This will include an immunofluorescence test.
• Blood test: Your provider will examine a small sample of your blood to check for immune system antibodies that cause your symptoms.

How is pemphigus foliaceus treated?

Treatment for pemphigus foliaceus is unique to each person diagnosed with the condition and could include:

• Stopping or changing any medicines that cause symptoms.
• Using corticosteroids (topical creams or oral medicines) or topical calcineurin inhibitors to reduce blistering.
• Taking immunosuppressive drugs like rituximab, methotrexate, mycophenolate or azathioprine.

Are there side effects of the treatment?

Every treatment option has different risks, so discuss the side effects with your provider before beginning your treatment. Ask your provider questions about when and how often you should take new medicines and whether or not that medicine will interact with any drugs you currently take. Once treatment begins, your provider will follow up with you a couple of weeks after your diagnosis to make sure treatment is effective. If your symptoms get worse or don’t improve after several weeks, talk to your provider.

How soon after treatment will I feel better?

Everyone’s body takes a different amount of time to heal. On average, you might notice new blisters stop forming after two weeks and the sores on your skin will start to heal. It could take several weeks for all of your sores to clear up on your skin. Symptoms could flare or arise again in the future since pemphigus is a chronic condition.

How can I prevent pemphigus foliaceus?

While you can’t prevent all causes of pemphigus foliaceus, you can take steps to reduce your risk of a flare by:

• Avoiding sun exposure or wearing protective clothing and sunscreen when in the sunlight.
• Talking to your provider about the medicines you currently take to see if they cause your symptoms. Don’t stop taking a medicine until your provider tells you it’s safe to do so.
• Wearing protective clothing, using insect screens and insect repellent to avoid insect bites in endemic areas.
• Using gentile or unscented soaps and lotions to soothe your skin.

What can I expect if I have pemphigus foliaceus?

There is no cure for pemphigus foliaceus. Symptoms of pemphigus foliaceus don’t interfere with your life expectancy. The condition can cause painful and itchy blisters and sores on your skin that heal with treatment. Blisters rarely cause scars. An injury or frequent scratching of a blister or a sore could lead to scarring.

When should I see my healthcare provider?

Visit your healthcare provider if you have:

• Blisters or sores that won’t heal.
• Blisters on a large part of your body.
• Sores that get bigger (swelling), leak a yellow or white fluid or have a yellow crust around them.
• A fever, chills or muscle aches.

What questions should I ask my doctor?

• What type of treatment do you recommend?
• Are there side effects to the treatment?
• What caused my symptoms?
• How often should I apply topical creams or ointments to my sores?

What is the difference between Pemphigus foliaceus and pemphigus vulgaris?

Pemphigus foliaceus is related to pemphigus vulgaris. Both conditions appear similar and pemphigus foliaceus could be in a differential diagnosis for pemphigus vulgaris.

Pemphigus vulgaris affects both your skin and your mucous membranes, which are the soft lining of the cavities in your body, which includes your mouth, throat and genitals. Pemphigus foliaceus doesn’t affect your mucous membranes and the symptoms are usually less severe.

Since pemphigus vulgaris can affect the mucous membranes in your mouth, you may need to adjust your diet so that your foods are bland and soft to avoid irritating the blisters in your mouth. There is no special diet for people diagnosed with pemphigus foliaceus since it doesn’t hinder your ability to eat.

A note from Cleveland Clinic

Pemphigus foliaceus is a skin condition that can cause painful and itchy blisters and sores on your skin. Symptoms of this condition can flare throughout your life unexpectedly but are often triggered by certain medicines or sunlight. Your provider will help you manage your symptoms and prevent infections. Be patient for your body to heal and try not to injure your blisters or skin sores. If you notice a yellow or white fluid or a yellow crust forming around your blister along with swelling and pain on your skin, contact your provider.