What is Pemphigus?

Pemphigus is the name for a group of autoimmune diseases. For reasons doctors don’t quite understand, these conditions cause your body’s natural defense system to attack itself.

If you have pemphigus, your immune system tries to destroy your skin and mucous membranes -- the moist parts of your body. What happens next is that you can get large blisters in your mouth, nose, throat, eyes, and genitals.

Pemphigus isn’t contagious. Luckily, it can be treated with medications.

What Are the Symptoms of Pemphigus?

There are two main types of this condition. What symptoms you have will depend on what type you have.

Pemphigus Vulgaris. This is the most common form. It affects the moist parts of your body, like your mouth and genitals. Adults between ages 30 and 60 are most likely to get it.

The first sign of a problem will typically be blisters in your mouth that peel easily. You may find it hard to swallow or eat.

Next, blisters will often form on your skin or inside your genitals. They hurt, but they don’t itch.

Pemphigus Foliaceus. These are crusty blisters that tend to form on your chest, back, and shoulders. They don’t hurt, but they do itch.

How is Pemphigus Diagnosed?

It can be tricky. That’s because a number of conditions can cause blisters. To make sure he finds the right cause, your doctor will likely order a number of tests, including:

  • A skin exam. He’ll use his finger or a cotton swab to rub a patch of your skin that’s not covered by a blister. If it peels easily, that could mean that you have pemphigus.
  • Skin biopsy. Your doctor will take a piece of tissue from one of your blisters and look at it under a microscope.
  • Blood tests. Your doctor will check your blood for specific antibodies called desmogleins. Antibodies are proteins your body makes for one, simple purpose: to find bad germs and kill them before they harm you.

If you have pemphigus, you’ll have more of these antibodies in your blood than normal. That’s because they’re banding together to fight bad germs. As your symptoms improve, the number of these antibodies in your blood goes down.

  • Endoscopy. If you have blisters in your mouth, your doctor may use a thin, flexible tube called an endoscope to look down your throat.

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Is It Pemphigus or Something Else?

Some skin diseases can look like pemphigus, but are actually different. Take bullous pemphigoid, for example. It causes large, fluid-filled blisters, but they generally don’t burst as easily. And unlike pemphigus, it usually affects people over age 60.

Herpes is another condition that can cause painful blisters on your mouth and genitals. But it’s not caused by your body attacking itself.

If you have itchy or painful blisters anywhere on your body, don’t try to figure out what’s wrong. See your doctor. Only he can tell you for sure whether you have pemphigus or something else.

What’s the Treatment for Pemphigus?

Your doctor will likely give you medicine that’ll help ease your symptoms and make you more comfortable. What he prescribes will depend on what type of pemphigus you have and how bad your symptoms are. Treatment might include:

  • Corticosteroids. These are usually the first line of treatment and can be very effective to relieve symptoms, often within a couple of weeks. They are typically given in pill form.
  • Immunosuppressants. These drugs keep your immune system from attacking healthy tissues.
  • Biological therapies. If other medications aren’t working, your doctor may give you a medication called rituximab (Rituxan). He’ll give it to you as an intravenous injection. It helps decrease the number of antibodies that are attacking your body.
  • Antibiotics, antivirals, and antifungal medications. These help to fight or prevent infections.

If pemphigus isn’t treated, it could be life-threatening. Sometimes, you may have to be admitted to the hospital until you get better.

At least 75% of people with pemphigus will have a complete remission, or no evidence of the disease, after 10 years of treatment. Some people must take medications for the rest of their lives to keep pemphigus symptoms from coming back.

WebMD Medical Reference Reviewed by Stephanie S. Gardner, MD on March 06, 2019

Sources

SOURCES:

National Institute of Arthritis and Musculoskeletal and Skin Diseases: “Question and Answers About Pemphigus.”

Mayo Clinic: “Pemphigus.”

U.S. National Library of Medicine, National Institutes of Health: “Exploring the Link Between Herpes Viruses and Pemphigus Vulgaris: Literature Review and Commentary.”

International Pemphigus & Pemphigoid Foundation: “Understanding P/P.”

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