What is it?

What is pemphigus?

Pemphigus is a rare disease that causes blistering on many parts of the body, including  the skin and the inside of the mouth, nose, throat, eyes, and genitals.

In pemphigus, the immune system mistakenly attacks cells in the top layer of the skin.

Who gets it?

Who gets pemphigus?

You are more likely to get pemphigus if you have certain risk factors. These include:

  • Ethnic background. Some populations, are at greater risk for certain types of the disease.
  • Geographic location. Certain types of pemphigus is more common in some places.
  • Sex and age. Depending on the type of pemphigus, sex and age can be a risk factor for the disease.
  • Genes. Doctors believe that genes may explain why certain places have more people with the disease.  
  • Medications. In rare cases, pemphigus has resulted from taking certain medicines.
  • Cancer. Rarely, a tumor can trigger the disease.
What are the types?

What are the types of pemphigus?

There are many forms of pemphigus. The two main forms are:

  • Pemphigus vulgaris is the most common type in the United States. Blisters form in the mouth and inside of the mouth, nose, throat, eyes, and genitals, as well as on the skin. They are often painful.
  • Pemphigus foliaceus is less common and only affects the skin. The blisters may be itchy or painful.
What are the symptoms?

What are the symptoms of pemphigus?

The main symptom of pemphigus is blistering of the skin. In some cases, other areas of the body may get blisters, including:

  • The inside of the mouth or nose.
  • Throat.
  • Eyes.
  • Genitals.

The blisters may burst, causing crusty sores that may become infected.

What causes it?

What causes pemphigus?

Pemphigus happens when your immune system attacks healthy skin, forming blisters.

Doctors do not know what causes the immune system to turn on the body’s own proteins, but they believe that both your genes and environmental factors are involved.

Is there a test?

Is there a test for pemphigus?

If you have blisters on the skin or in the mouth that do not go away, it is important to see a doctor as soon as you can. Your doctor may:

  • Take your medical history, and give you a physical exam. 
  • Take a tissue sample from one of your blisters. 
  • Take a blood sample.
How is it treated?

How is pemphigus treated?

There is no cure for pemphigus, but treatment can control the disease in most people. The goal of treatment is to clear existing blisters and help prevent relapses. Your doctor may prescribe medications to treat the disease. 

Symptoms of pemphigus may go away after many years of treatment, but most people need to keep taking medications to keep the disease under control.

In some cases, a person with pemphigus may need to be treated in a hospital for health problems that the disease can cause. Having many sores on the skin can result in dehydration or infection, and painful blisters in the mouth can make it difficult to eat. In the hospital, you may be given an IV to replace lost fluids, to get nutrition, and to treat infection.

Who treats it?

Who treats pemphigus?

The following health care providers may diagnose and treat pemphigus:

  • Dermatologists, who specialize in conditions of the skin, hair, and nails.
  • Dentists, who can tell you how to take care of your gums and teeth if you have blisters in your mouth.
  • Mental health professionals, who help people cope with difficulties in the home and workplace that may result from their medical conditions. 
  • Ophthalmologists, in cases where the eyes are affected. Ophthalmologists specialize in treating disorders and diseases of the eye. 
  • Primary care doctors, such as a family physician or internal medicine specialist, who coordinate care between the different health care providers and treat other problems as they arise.
Living With It

Living with pemphigus

Living with pemphigus can be difficult, but there are things you can do to cope.

  • If blisters in your mouth may make brushing and flossing your teeth painful, talk to your dentist about ways to keep your teeth and gums healthy.
    • Avoid foods that bother your mouth blisters.
  • Take baths and use wound dressings if your doctor recommends them to help heal the sores and blisters.
  • Visit a mental health professional or join a support group if you experience stress related to pemphigus, especially from effects such as:
    • Lost time at work.
    • Sleep problems.
    • Emotional distress.

Remember to follow the recommendations of your health care providers.

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