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Pemphigus

Overview

Pemphigus is a group of rare skin disorders that cause blisters and sores on the skin or mucous membranes, such as in the mouth or on the genitals.

The two main types are pemphigus vulgaris and pemphigus foliaceus. Pemphigus vulgaris usually starts in your mouth. It can be painful. Pemphigus foliaceus affects the skin and tends to be more itchy than painful. Pemphigus can occur at any age, but it's most often seen in people who are middle-aged or older.

Pemphigus is not to be confused with bullous pemphigoid, another blistering skin condition. Usually a chronic condition, pemphigus is best controlled by early diagnosis and treatment. Treatment may include medications and therapies similar to those used for severe burns.

Pemphigus vulgaris

Pemphigus is a rare skin disorder characterized by blistering of your skin and mucous membranes. The most common type is pemphigus vulgaris, which involves painful sores and blisters on your skin and in your mouth.

Pemphigus vulgaris

Pemphigus is a rare skin disorder characterized by blistering of your skin and mucous membranes. The most common type is pemphigus vulgaris, which involves painful sores and blisters on your skin and in your mouth.

Pemphigus foliaceus

Pemphigus foliaceus doesn't usually affect mucous membranes. The blisters, which usually begin on your face and scalp and later erupt on your chest and back, usually aren't painful. They tend to be crusty and itchy.

Symptoms

Pemphigus is characterized by blisters on your skin and mucous membranes. The blisters rupture easily, leaving open sores, which may ooze and become infected.

The signs and symptoms of the two main types of pemphigus are as follows:

  • Pemphigus vulgaris. This type usually begins with blisters in your mouth and then on your skin or genital mucous membranes. The blisters typically are painful, but don't itch. Blisters in your mouth or throat may make it hard to swallow and eat.
  • Pemphigus foliaceus. This type doesn't usually affect mucous membranes. And the blisters tend not to be painful. This condition may affect any skin, but most blisters are on the chest, back and shoulders. The blisters cause the skin to be crusty and itchy.

When to see a doctor

See your doctor if you develop blisters inside your mouth or on your skin. If you've already been diagnosed with pemphigus and are receiving treatment, see your doctor if you develop:

  • New blisters or sores
  • A rapid spread in the number of sores
  • Fever, redness or swelling, which may indicate infection
  • Chills
  • Weakness or achy muscles or joints

Causes

Pemphigus is an autoimmune disorder. It's not contagious. In most cases, it's unknown what triggers the disease.

Normally, your immune system attacks foreign invaders, such as harmful viruses and bacteria. But in pemphigus, your immune system mistakenly produces antibodies that attack healthy cells in your skin and mucous membranes.

Rarely, pemphigus develops as a side effect of medications, such as certain blood pressure drugs. This type of pemphigus usually disappears when the medicine is stopped.

Risk factors

Your risk of pemphigus increases if you're middle-aged or older. People of Jewish ancestry have an increased incidence of pemphigus vulgaris.

Complications

The open sores of pemphigus make you highly vulnerable to infection, which, if it spreads to your bloodstream, can be fatal. Possible complications of pemphigus include:

  • Infection of your skin
  • Infection that spreads to your bloodstream (sepsis)
  • Gum disease and tooth loss, if you have blisters in your mouth
  • Medication side effects, such as high blood pressure and infection
  • Death from infection

Diagnosis

Blisters occur with a number of conditions, so pemphigus can be difficult to diagnose. Your doctor will ask you for a complete medical history and examine your skin and mouth. In addition, he or she may:

  • Check for skin peeling. Your doctor will lightly rub a patch of normal skin near the blistered area with a cotton swab or finger. If you have pemphigus, the top layers of your skin are likely to shear off.
  • Do a skin biopsy. In this test, a piece of tissue from a blister is removed and examined under a microscope.
  • Run blood tests. One purpose of these tests is to detect and identify antibodies in your blood known as desmogleins. These antibodies are often elevated when pemphigus is first diagnosed. The levels of these antibodies usually goes down as symptoms improve.
  • Order an endoscopy exam. If you have pemphigus vulgaris, your doctor may have you undergo endoscopy to check for sores in the throat. This procedure involves inserting a flexible tube (endoscope) down your throat.

Treatment

Treatment usually begins with medications that are intended to reduce signs and symptoms and prevent complications. It's generally more effective when it begins as early as possible. Treatment may also involve a hospital stay. Pemphigus may be life- threatening.

Medications

The following prescription medications may be used alone or in combination, depending on the type and severity of your pemphigus:

  • Corticosteroids. For people with mild disease, corticosteroid cream may be enough to control it. For others, the mainstay of treatment is corticosteroids, such as prednisone pills.

    Using corticosteroids for a long time or in high doses may cause serious side effects, including increased blood sugar, bone loss, an increased risk of infection, cataracts, glaucoma and a redistribution of body fat, leading to a round face (moon face).

  • Immunosuppressants. Medications such as azathioprine (Imuran) or mycophenolate mofetil (CellCept) help keep your immune system from attacking healthy tissue. They may have serious side effects, including increased risk of infection.
  • Biological therapies. Your doctor may suggest a drug called rituximab (Rituxan) if other medications aren't helping or are difficult for you to tolerate. This drug is given as an injection. It targets the white blood cells responsible for the production of the pemphigus antibodies.
  • Antibiotics, antivirals and antifungal medications. These may be used to control or prevent infections.
  • Other medications. Other drugs that alter the immune system may be effective. These include dapsone and intravenous immunoglobulin.

Hospital stay

Some therapies for pemphigus may require a hospital stay. Along with medications listed above, you may be given:

  • Fluids. Because skin sores can result in significant loss of fluid from your body, replacing fluids may be an important part of treatment. You may receive fluids through a vein (intravenously).
  • Intravenous feeding. This may be necessary if mouth sores make it too painful for you to eat. You may receive fluids and nutrients through a tube placed through your nose and advanced into your stomach (nasogastric tube) until normal nutrition can be restored.
  • Anesthetic products for the mouth. These can help control pain of mild to moderate mouth sores.
  • Therapeutic plasmapheresis. In this process, the fluid part of your blood, called plasma, is removed from blood cells by a device known as a cell separator. The purpose is to get rid of the antibodies that are attacking your skin. The plasma is replaced with donated plasma or intravenous fluids.
  • Wound care. You may be given gentle baths and dressings to help your blisters and sores heal.

Many people get better with treatment, although it may take years. Others need to take a lower dose of medication indefinitely to prevent their signs and symptoms from returning.

Preparing for an appointment

You're likely to first see your primary care doctor. He or she may refer you to a doctor who specializes in skin disorders (dermatologist).

Here's some information to help you get ready for your appointment.

What you can do

Before your appointment make a list of:

  • Symptoms you've been having and for how long
  • Key personal information, including any major stresses or recent life changes
  • All medications, vitamins and supplements you take, including doses
  • Questions to ask your doctor

For pemphigus, some basic questions to ask your doctor include:

  • What's the most likely cause of my symptoms?
  • Are there other possible causes?
  • Do I need any tests? Do these tests require any special preparation?
  • What treatments are available, and which do you recommend?
  • What side effects can I expect from treatment?
  • How long will it take for the blisters to heal? Will they leave scars?
  • Will the blisters come back again?
  • What can I do for the pain?
  • I have these other health conditions. How can I best manage them together?
  • Is there a generic alternative to the medicine you're prescribing me?
  • Do you have any brochures or other printed material I can take with me? What websites do you recommend?

What to expect from your doctor

Examples of questions your doctor may ask, include:

  • When did you first begin experiencing symptoms?
  • Does anything seem to improve your symptoms?
  • What steps have you taken to treat this condition yourself?
  • Have any of these measures helped?
  • Have you ever been treated by a doctor for this condition?
  • If so, did you use any prescription treatments for this skin condition? If so, do you remember the name of the medication and the dosage you were prescribed?
  • Did you have a skin biopsy?

Lifestyle and home remedies

Here are steps you can take to improve your skin and overall health:

  • Follow your doctor's wound care instructions. Taking good care of your wounds can help prevent infection and scarring.
  • Use talcum powder. Generously sprinkling talcum powder on your sheets may help keep oozing skin from sticking.
  • Take your medicines as prescribed. Stopping or changing the dose could cause your condition to worsen.
  • Clean towels, linens and clothing. Frequent cleaning of these items helps prevent your blisters and sores from becoming infected. Also, don't share such items with others.
  • Protect your skin. Avoid activities that may hurt or contaminate your wounds, such as playing contact sports or using a hot tub.
  • Avoid certain foods. Blisters in your mouth could be triggered or irritated by some foods, such as garlic, onion and abrasive foods.
  • Minimize sun exposure. Ultraviolet light may trigger new blisters.
  • Talk with your dentist about maintaining good oral health. If you have blisters in your mouth, it may be difficult to brush your teeth properly. Ask your dentist what you can do to protect your oral health.
  • Ask your doctor if you need calcium and vitamin D supplements. Corticosteroids can affect your calcium and vitamin D needs, so ask your doctor if you need a calcium supplement or other additional nutrients.

Coping and support

Pemphigus may be difficult to live with, especially if it affects your daily activities or makes you feel embarrassed. You may find it helpful to talk to others with the disease. You can find in-person or virtual support groups. Ask your doctor for suggestions.

Last updated: November 18th, 2015

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