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

There are two main types: pemphigus vulgaris and pemphigus foliaceus. Pemphigus vulgaris is the most common form. Pemphigus can occur at any age, but often strikes people in middle age or older.

Usually a chronic condition, pemphigus is best controlled by early diagnosis and treatment, which may include medications or treatments similar to those used for severe burns.

Symptoms Causes Risk factors Complications

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 main types of pemphigus differ depending on the type:

  • Pemphigus vulgaris. The most common form, pemphigus vulgaris usually begins with blisters in your mouth, which then erupt on your skin. Blisters can also break out on the mucous membranes of your genitals. The blisters typically are painful, but don't itch. Blisters in your mouth or throat may make it hard to swallow and to eat.
  • Pemphigus foliaceus. This type 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.

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 any of the following:

  • New blisters or sores
  • A rapid spread in the number of sores
  • Fever
  • Chills
  • Achy muscles or joints

Pemphigus is an autoimmune disorder, and 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.

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

Pemphigus isn't contagious, and there's no way to predict who'll get it. However, your risk increases if you're middle-aged or older.

Possible complications of pemphigus include:

  • Infection of your skin
  • Infection that spreads through your bloodstream (sepsis)
  • Medication side effects, such as an increased risk of infection
  • Rarely, death from infection
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