Pemphigus Vulgaris

What is pemphigus vulgaris?

Pemphigus vulgaris is an autoimmune condition that causes blisters to form on your skin and mucous membranes. Mucous membranes are the soft lining that covers the surfaces of internal organs and cavities in your body, especially around your respiratory, digestive, urinary and genital tracts. Most people diagnosed with pemphigus vulgaris have blisters in their mouth. These blisters can break open easily and turn into painful sores.

Pemphigus vulgaris is the most common form of pemphigus.

How common is pemphigus vulgaris and who does it affect?

Pemphigus vulgaris is rare and can affect anyone, but it most often affects people over the age of 40. Pemphigus vulgaris rarely affects babies and children.

How does pemphigus vulgaris affect my body?

Symptoms of pemphigus vulgaris usually arise in your mouth, which causes painful blisters to form that can make eating and drinking difficult. After targeting the mucous membranes in your mouth, your symptoms can move to the skin on other parts of your body. These blisters are very thin and can easily break open. When your blisters break, they can leak fluid and cause your skin to peel, which turns the blisters into open sores.

What are the symptoms of pemphigus vulgaris?

Symptoms range in severity for each person diagnosed with pemphigus vulgaris and could include:

• Fluid-filled bump (blister) that has a red halo around the outside and a light pink to white center.
• The blister breaks open and leaks a clear fluid or lightly bleeds.
• Crust or scales form on or around the blister.
• The skin on or around the blister peels off.
• Open sores form when a blister breaks.
• Pain on or around the blisters.

Be careful not to injure or scratch your blisters, as they become open sores or wounds that can easily become infected. Signs of an infection include:

• White or yellow pus leaks out of the blister.
• The blister burns or you have severe pain.
• A yellow crust forms on or around the blister.
• The skin around your blister swells or gets bigger.
• Your blister doesn’t heal.

Where will I have symptoms of pemphigus vulgaris?

Symptoms of pemphigus vulgaris affect your skin and your mucous membranes. The most common places where you’ll experience symptoms include:

• Mouth and throat.
• Nose.
• Eyes.
• Genitals.
• Chest.
• Back.
• Arms and legs.
• Scalp.

What causes pemphigus vulgaris?

The exact cause of pemphigus vulgaris is unknown.

Since pemphigus vulgaris is an autoimmune condition, antibodies in your immune system, which are proteins that protect your body from foreign invaders like bacteria and toxins, target healthy cells and mistake them for foreign invaders. This attack on your healthy cells causes symptoms of blistering on your skin and mucous membranes.

In rare cases, your body can negatively react to certain medicines that cause symptoms of pemphigus vulgaris, including:

• ACE inhibitors: Blood pressure medicines.
• Chelating agents: Medicines to remove specific materials from your blood.
• Antibiotics.
• Nonsteroidal anti-inflammatory drugs (NSAIDs).

Is pemphigus vulgaris contagious?

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

How is pemphigus vulgaris diagnosed?

Your healthcare provider will diagnose pemphigus vulgaris after a physical exam to learn more about your symptoms and will ask questions about your health and medical history. They’ll also offer tests to diagnose your condition.

What tests diagnose pemphigus vulgaris?

To confirm a diagnosis, your healthcare provider might offer a skin biopsy. During a skin biopsy, your healthcare provider will remove a small sample of your tissue from an affected area of your skin to examine it under a microscope. This may include an immunofluorescence test.

Another test is a blood test to check for immune system antibodies that cause your symptoms.

How is pemphigus vulgaris treated?

Treatment for pemphigus vulgaris focuses on managing symptoms and preventing complications like infections. Treatment is unique to each person and could include:

• Taking medicines to treat infections.
• Stopping any medicines that cause symptoms.
• Using medicines, creams or ointments to treat, soothe and heal sores.
• Caring for sores and broken blisters as you would a burn or wound.
• Eating a bland diet or receiving nutrients through an IV to avoid malnutrition if sores in your mouth prevent you from eating.
• Using numbing anesthetic medicine to minimize pain from blisters in your mouth.

What medications treat pemphigus vulgaris?

• Your healthcare provider might recommend taking medicines to reduce your symptoms, prevent flares of symptoms or treat infections. Medicines could include topical or oral corticosteroids.
• Immune system suppressants (azathioprine, cyclosporine, mycophenolate, cyclophosphamide, methotrexate or rituximab).
• Antibiotics or antifungals.

Are there side effects of the treatment?

Each treatment is different and there could be side effects depending on what medicine your healthcare provider prescribes. Before starting a new medicine, tell your healthcare provider about any medicines or supplements you actively take and about any allergies you have. They’ll monitor the progress of your condition and treatment by offering routine blood or urine tests to make sure you don’t have any side effects.

What can I eat or drink with pemphigus vulgaris?

Eating and drinking can be challenging with pemphigus vulgaris, especially if you have blisters and sores in your mouth. Choose foods that are soft, liquid and bland. These will cause the least amount of irritation to your sores. Avoid foods that are acidic, spicy and crunchy, like citrus fruits, hot wings or potato chips.

If you can’t eat or drink, contact your healthcare provider.

How soon after treatment will I feel better?

The length of time you need to heal varies for each person. On average, it could take several weeks for new blisters to stop forming after you start treatment. After that, it could take several months for your skin to heal.

Pemphigus vulgaris is a chronic condition, which means that your symptoms can return after you receive treatment.

How can I prevent pemphigus vulgaris?

You can’t prevent pemphigus vulgaris because the cause is unknown. Treatment is effective to reduce your symptoms.

What can I expect if I have pemphigus vulgaris?

With treatment, you can control pemphigus vulgaris. The condition rarely affects your life expectancy. Symptoms can be temporarily disabling if they’re severe, which can prevent you from going about your routine or going to work. Some people have trouble sleeping and may experience weight loss if the sores in their mouth prevent them from eating normally.

Blisters rarely cause scars when they heal, but if a sore breaks open and has trouble healing, you might have scarring on your body or discolored marks on your skin.

Without treatment, symptoms can spread throughout your body and cause life-threatening complications like malnutrition, dehydration and sepsis. A severe, untreated infection is the most common complication of untreated pemphigus vulgaris.

There isn’t a cure for pemphigus vulgaris. Studies are ongoing to learn more about the cause and new treatment options to alleviate symptoms.

How long does pemphigus vulgaris last?

Pemphigus vulgaris is a chronic condition and you could experience symptoms throughout your life. Most often, symptoms will begin between the ages of 40 to 60. Symptoms can unexpectedly arise (flare) and go away. Treatment can help reduce your symptoms.

How do I take care of myself?

You can take care of yourself and manage your symptoms of pemphigus vulgaris at home by:

• Eating a bland, smooth and liquid-based diet when you have active sores in your mouth.
• Cleaning and caring for your blisters and sores like wounds or burns.
• Protecting your skin from the sun’s UV rays by wearing protective clothing and sunscreen.
• Using gentle soaps or unscented lotions on your skin.

When should I see my healthcare provider?

Visit your healthcare provider if you have:

• Sores that won’t heal.
• Blisters on a large part of your body.
• Painful blisters that ooze a yellow or white fluid and form a yellow crust around them.
• Swelling on or near your blisters.
• Trouble eating and drinking.
• 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?
• How do I prevent infections?
• How do I care for my sores at home?

What’s the difference between pemphigus vulgaris and bullous pemphigoid?

Both pemphigus vulgaris and bullous pemphigoid are autoimmune conditions that affect your skin and target people older than 65 years. The major difference between both conditions is the lesions and cause.

Pemphigus vulgaris causes small, fluid-filled blisters to form on your skin and in your mucous membranes, like in your mouth. Bullous pemphigoid causes itchy, hive-like welts on your skin. These welts often have intact, tense blisters.

A note from Cleveland Clinic

Pemphigus vulgaris is a persistent skin condition that you’ll have throughout your life. Symptoms can be uncomfortable and cause sores on your skin and in your mouth. Make sure you’re eating and drinking during your diagnosis to prevent malnutrition. Talk to your healthcare provider about treatment options unique to your symptoms.