Health Encyclopedia

Pemphigus Vulgaris

Pemphigus is a rare group of autoimmune diseases that causes blisters on the skin and mucous membranes--mouth, nose, throat, eyes, and genitals--throughout the body. Pemphigus vulgaris is the most common type of pemphigus.

Pemphigus vulgaris is not fully understood, but experts believe that it's triggered when a person who already has a genetic tendency to develop this condition comes into contact with an environmental "switch," such as a chemical or a drug. In some cases, pemphigus vulgaris will go away once the environmental trigger is removed.

Certain ethnic groups, including people of eastern European Jewish and Mediterranean descent, are more prone to this condition.

As an autoimmune condition, pemphigus causes the immune system to fight against the body's own cells in the same way that it fights off invading germs.

Anatomy of the skin
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With pemphigus vulgaris, the immune system targets proteins that bind the cells of the skin. This causes a buildup of fluid between the skin cells, resulting in blisters. Experts believe that in addition to fighting healthy proteins, pemphigus vulgaris interferes with the cellular signaling that helps the skin stay healthy.

Symptoms

Pemphigus vulgaris typically begins in the mouth. Symptoms include:

  • Blisters on otherwise healthy skin

  • Blisters that are easy to burst

  • Affected skin that peels easily when rubbed

  • Pain at blister

Complications

Blisters heal, but may be painful and leave dark patches on the skin for months. Most people with pemphigus vulgaris can find relief with treatment. Without treatment, the condition can lead to severe pain, infection, and even death.

When to call a doctor

Call your doctor if you notice painful, soft blistering on your skin or mucous membranes. Treatment will prevent the blisters from spreading and getting worse.

Diagnosis

You may need to see a dermatologist to diagnose and treat this condition. Your doctor visit may include:

  • Taking your medical history

  • Physical exam

  • Review of your symptoms

  • Blood tests

  • Biopsy, or taking a tissue sample for testing

Treatment

These are common treatments for pemphigus vulgaris:

  • Better oral health care. Because blistering may affect the health of your mouth, work with your dentist to be sure that you're taking the best care of your teeth and gums.

  • Steroid medications. Prednisone and steroid creams may be prescribed to treat the inflammation.

  • Immune suppressants. As an alternative to steroid treatments, immune-suppressing medicines may be helpful in managing this disease.

  • Plasmapheresis and/or intravenous immunoglobulin. People whose pemphigus vulgaris does not respond to other forms of treatment may need more intensive treatments, including the replacement of blood plasma and infusions with healthy immunoglobulin.

  • Lifestyle management. Some people find that stress and certain foods, such as garlic, make living with pemphigus vulgaris more difficult, even during treatment. Pay attention to what helps you feel better and what seems to make symptoms worse.

  • Follow-up. This condition may return after successful treatment. Go to all follow-up appointments.

It can take two to five years or even longer to treat this condition. Also, treatments for pemphigus vulgaris may have serious side effects. Talk with your doctor about potential side effects and how to manage them. 



Medical Reviewers:

  • Sohrabi, Farrokh, MD
  • Ziegler, Olivia Walton, MS, PA-C