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What Is Pemphigus?

This is a rare autoimmune skin disorder that occurs when your immune system starts attacking healthy epidermis cells (the top layer of your skin). Pemphigus can manifest itself as blisters and sores on your skin or mucous membranes of the body like your mouth, eyes, nose, throat, and genitals. The blisters rupture with ease, which leave open, oozing sores that may become infected.

Pemphigus Types

The two most common types of pemphigus are:

  • Pemphigus vulgaris — This begins with painful blisters in your mouth that do not itch. The blistering may then spread to other parts of your body or genital mucous membranes.
  • Pemphigus foliaceus — These itchy blisters form on your back, chest and shoulders, but not in your mouth. They are not usually painful either.

Other types of the disease include:

  • Pemphigus vegetans — Thick sores in your groin and armpits are a tell-tale sign of this kind of pemphigus.
  • IgA pemphigus — This is the least harmful type of pemphigus that can cause small, pus-fillled bumps. The blisters bear a striking resemblance to pemphigus foliaceus, but they are actually caused by the IgA antibody.
  • Paraneoplastic pemphigus — This is a rare form of pemphigus that can occur in people with certain types of cancer. Symptoms may include painful mouth and lip sores, cuts and scars on the lining of the eyes and eyelids, blisters, and severe lung problems.

Pemphigus Symptoms

The hallmark signs of pemphigus include the following:

  • blisters that come and go or rupture and cause infection,
  • sores on the skin or in the mucous membranes, like the mouth, throat, nose, eyes, or genitals,
  • crusting and oozing at the blister site, and
  • presence of the Nikolsky sign, a test that rubs the top layer of your skin to determine how easily it slips away from the underlying skin layers.
A person's back with pemphigus blisters

Pemphigus Causes

Doctors still do not know what causes your immune system to mistake healthy cells for viruses or harmful bacteria. But we do know that It is not contagious. It cannot be passed down from parent to child either. However, your genes may make you more susceptible to developing pemphigus than others.

Find an Autoimmune Skin Disease Specialist

Pemphigus vs. Pemphigoid

Pemphigoid is another autoimmune skin disorder that bears a striking resemblance to pemphigus because blisters and rashes can appear on the skin and mucous membranes of the body. One of the most prominent distinctions between the two diseases is that pemphigoid blisters embed themselves deep into the skin, which means they do not break easily. Another difference is that pemphigoid occurs under the top layer of the skin.

Types of Pemphigoid

There are three kinds of pemphigoid which include:

  • Bullous pemphigoid — The most common type of pemphigoid that causes rashes and blisters to appear on the arms, legs, joints or lower abdomen.
  • Cicatrical pemphigoid — This form of pemphigoid causes blisters to form on the mucous membranes.
  • Pemphigoid gestationis — This blistering occurs during or shortly after pregnancy.
Pemphigoid blisters

Pemphigoid Symptoms

The following signs of pemphigoid are:

  • rashes and fluid-filled blisters,
  • thick-walled blisters,
  • blisters that form on mucous membranes, especially the mouth, eyes, nose, throat, or genitals,
  • arm and leg blisters that commonly form on areas where movement occurs, and
  • absence of the Nikolsky sign, which tests how easily the top layer of skin is removed from the bottom layers.

Diagnostic Procedures

Pemphigus and pemphigoid are both rare and blisters can be a sign of many different health conditions so we may test you for other diseases first. During your initial visit, our autoimmune skin disease specialist will go over your medical history and conduct a physical examination. We will then remove a piece of the blister and inspect it under a microscope. We can determine the type of pemphigus by running on the skin sample. We may also draw your blood to measure the levels of pemphigus antibodies in your system.

If your doctor suspects you have pemphigus vulgaris or bullous pemphigoid, an endoscopy may be performed to check for sores in your throat. This procedure involves sticking a flexible tube (endoscope) down your throat to see deep down inside.

Pemphigus & Pemphigoid Treatment

We may not know what causes these autoimmune disorders, however, there are treatments that can assist in easing your symptoms:

  • Topical medication (eg. corticosteroid creams)
  • with anti-inflammatory and immunosuppressant ingredients
  • Medication infusions administered through an IV tube
  • Wound care

It can take months or years of treatment for people to get better while others may need to take medication for the rest of their life to keep their symptoms at bay. Some may even be hospitalized for severe or infected sores.

Disease Prognosis

Once someone starts treatment, they can typically live a long and healthy life. However, pemphigus and pemphigoid can become potentially life-threatening diseases, if left untreated. You can greatly improve and possibly eliminate your symptoms by following your doctor’s treatment plan. It could even increase your likelihood of entering into remission (no evidence of disease).

Who Is At Risk?

People over the age of 60 have a greater risk of developing these diseases. People who are in poor health or do not seek treatment are more likely to die from these diseases. However, anyone can get pemphigus or pemphigoid at any age. You could even get pemphigoid while pregnant.  

Make An Appointment with Our Specialists

A referral is required to meet with an autoimmune skin disease specialist. However, if you have questions about a skin condition without a pre-existing diagnosis, you can schedule an appointment with a general dermatologist at the U of U Health clinic by contacting 801-581-2955.

For new patients with an existing diagnosis, you will need a referral from your current provider. Your referral should include your name, date of birth, home address, phone number, and insurance company.

Before your first appointment, records about your autoimmune history should be faxed to U of U Health at 801-581-4911. These records should include clinic notes, biopsy reports, lab reports, diagnostic studies, radiographic studies, and treatments.

Please make sure your doctor faxes your referral to:

University of Utah
Department of Dermatology
Attn: Autoimmune Clinic

Phone: 801-581-2955, ask for autoimmune scheduling
Fax: 801-581-4911

We will review referral requests within 48 hours to make sure that the best dermatologist evaluates each case. We will contact new patients about an appointment.