Pemphigus

Reviewed by Debra Jaliman, MD on July 13, 2020

What Is Pemphigus?

 

Pemphigus is the name for a group of autoimmune diseases. 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. That can cause large blisters in your mouth, nose, throat, eyes, and genitals.

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

Pemphigus Types and Symptoms

There are several types of this condition. Your symptoms 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.

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

Endemic pemphigus (fogo selvagem). This is a rare type of pemphigus foliaceus that happens most often in South America, especially Brazil. It often affects more than one member of the same family.

Pemphigus vegetans. This type is a lot like pemphigus vulgaris. But instead of blisters, it forms thick, wart-like lesions. These usually appear on parts of the body where your skin rubs against itself, like the armpit or groin.

Drug-induced pemphigus. If you have this type, certain medications will cause blisters that can form up to 6 months after you’ve taken them.

Pemphigus erythematosus (Senear-Usher syndrome). Blisters show up on your scalp, cheeks, upper back, and upper chest. They can turn into red, crusty lesions.

Paraneoplastic pemphigus. This is the rarest type of pemphigus. It affects people who have cancer. If you have it, you may have blisters in your mouth that you can’t treat. Treating the cancer will help to ease the blisters.

Causes

Pemphigus is an autoimmune disorder. This means that your immune system attacks your body’s cells -- in this case, the skin cells. In rare cases, certain drugs can trigger the condition. This includes penicillin and some rheumatoid arthritis medications. But most of the time, we don’t know what causes it. It isn’t contagious.

Who Does Pemphigus Affect?

Some people are more likely to develop this condition than others. This includes those who are:

  • Of Jewish ancestry
  • Of Indian, Middle Eastern, or Southeastern European descent
  • Are 40 years old and older
  • People with autoimmune diseases, especially the disease myasthenia gravis.

Pemphigus Diagnosis

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

Medical history. Your doctor will ask you what medications you take, as some drugs can cause this condition. 

A skin exam. Your doctor will use their finger or a cotton swab to rub a patch of your skin that’s not covered by a blister. If it peels easily, you could have pemphigus.

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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 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. 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.

Conditions With Similar Symptoms

Some skin diseases can look like pemphigus. Bullous pemphigoid, for example, 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 they can tell you for sure whether you have pemphigus or something else.

Pemphigus Treatment

Your doctor will likely give you medicine that’ll help ease your symptoms and make you more comfortable. What they prescribe 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). You'll get it in an injection. It helps decrease the number of antibodies that are attacking your body.

Antibiotics, antivirals, and antifungal medications. These 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.

Wound care. Topical antibiotics and wound dressings are used to heal the blisters.

Plasmapheresis.  If the pemphigus doesn’t respond to the other treatments, your doctor can use a special machine to take the plasma from your blood. They then replace it with plasma from a blood donor.

Pemphigus Complications

Several serious complications can happen with pemphigus. Among them:

  • Skin infections
  • Infections that get into the bloodstream (sepsis)
  • Being unable to eat, leading to weight loss and malnutrition
  • Depression
  • Some forms of the condition can cause death if not treated

The medications that you take for pemphigus can also cause serious side effects. They can include:

  • Infections
  • Rashes
  • High blood pressure
  • Low bone density (osteoporosis)

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

Sources

SOURCES:

Indian Journal of Dermatology: “Mental Health in Patients with Pemphigus: An Issue to Worth Consideration.”

International Pemphigus and Pemphigoid Foundation: “Complications & Side Effects.”

Cleveland Clinic: “Pemphigus.”

Johns Hopkins Medicine: “Pemphigus Vulgaris.”

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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