What is pemphigus vulgaris?

Pemphigus is a rare group of autoimmune diseases. It causes blisters on the skin and mucous membranes in the body. It can affect the mouth, nose, throat, eyes, and genitals. Pemphigus vulgaris is the most common type of pemphigus. It isn't contagious.

What causes pemphigus vulgaris?

Pemphigus vulgaris is not fully understood. Experts believe that it’s triggered when a person who has a genetic tendency to get this condition comes into contact with an environmental trigger. This might be a chemical or a medicine. In rare cases, pemphigus vulgaris will go away once a trigger is removed.

The condition causes the immune system to fight against the body’s own cells in the same way that it fights off germs.

With pemphigus vulgaris, the immune system starts to fight against the normal proteins that bind the cells of the skin together. This causes a buildup of fluid between the skin cells, resulting in blisters. Experts believe that the condition fights healthy proteins.

What are the symptoms of pemphigus vulgaris?

Pemphigus vulgaris often starts in the mouth. Symptoms include:

• Blisters on otherwise healthy skin

• Blisters that are easy to burst

• Affected skin that peels easily when rubbed

• Pain at blister

Who is at risk for pemphigus vulgaris?

Certain ethnic groups are more prone to this condition. This includes people of eastern European Jewish and Mediterranean descent. It's more common in people who have other autoimmune diseases like myasthenia gravis and thymoma. Pemphigus vulgaris is most often diagnosed in middle-aged and older people.

How is pemphigus vulgaris diagnosed?

You will likely need to see a dermatologist to diagnose and treat this condition. Your healthcare provider visit may include:

• Taking your health history

• Doing a physical exam

• Reviewing your symptoms

• Testing blood

• Taking a tissue sample of your skin for testing (biopsy)

How is pemphigus vulgaris treated?

These are common treatments for pemphigus vulgaris:

• Good oral healthcare. Blistering may affect the health of your mouth. So working with your dentist to take the best care of your teeth and gums is important.

• Oral prednisone and topical steroid creams. These may be prescribed to treat inflammation.

• Immune suppressants. These medicines may be a helpful alternative to steroids for long-term use.

• Plasmapheresis or intravenous immunoglobulin. People who do not respond to other forms of treatment may need more intensive treatments. This may include replacing blood plasma and infusions with healthy immunoglobulin.

• Pain management. Pain medicine or other pain management strategies may be used to help with painful blisters.

• Follow up. This condition may return, even after successful treatment. Go to all follow-up appointments.

You may be advised to use baths and special wound dressings to help sores heal. If you get an infection, your healthcare provider may prescribe antibiotic, antifungal, or antiviral medicines. People with severe cases of pemphigus vulgaris may need to be hospitalized to get wound care and intravenous (IV) fluids or electrolytes if mouth sores make it difficult to eat and drink.

It can take months or even years to treat and control this condition. Also, treatments may have serious side effects. Talk with your healthcare provider about possible side effects and how to manage them. 

What are possible complications of pemphigus vulgaris?

Blisters may be painful. They may heal and leave dark patches on the skin for months. Most people with pemphigus vulgaris feel better with treatment. Without treatment, the condition can lead to severe pain and infection.

When should I call my healthcare provider?

Call your healthcare provider if you notice painful, soft blistering on your skin or mucous membranes. Treatment will prevent the blisters from spreading, becoming infected, and getting worse.

Living with pemphigus vulgaris

Some people find that stress and eating certain spicy, acidic, or citrus foods make pemphigus vulgaris worse. This may be true even during treatment. Pay attention to what helps you feel better and what makes symptoms worse.

Key points about pemphigus vulgaris

• Pemphigus is a rare group of autoimmune diseases. It causes blisters on the skin and mucous membranes. It can affect the mouth, nose, throat, eyes, and genitals.

• Pemphigus vulgaris is the most common type of pemphigus. It often starts in the mouth.

• Symptoms include blisters that are painful and easy to burst.

• Experts believe that it’s triggered when a person who has a genetic tendency to get this condition comes into contact with a trigger.

• You may need to see a dermatologist to diagnose and treat this condition.

Next steps

Tips to help you get the most from a visit to your healthcare provider:

• Know the reason for your visit and what you want to happen.

• Before your visit, write down questions you want answered.

• Bring someone with to help ask questions and remember what your provider tells you.

• At the visit, write down the name of a new diagnosis and any new medicines, treatments, or tests. Also write down any instructions your provider gives you.

• Know why a new medicine or treatment is prescribed and how it will help. Also know what the side effects are and when they should be reported.

• Ask if your condition can be treated in other ways.

• Know why a test or procedure is recommended and what the results could mean.

• Know what to expect if you do not take the medicine or have the test or procedure.

• If you have a follow-up appointment, write down the date, time, and purpose for that visit.

• Know how you can contact your provider if you have questions or concerns. Ask how to contact your provider's office on weekends, evenings, and holidays.