Pemphigus

Causes

Pemphigus is an autoimmune disorder. This means that your immune system attacks your body’s cells — in this case, the skin cells. In rare cases, certain drugs can trigger the condition. This includes penicillin and some rheumatoid arthritis medications. But most of the time, we don’t know what causes it. It isn’t contagious.

Who Does Pemphigus Affect?

Some people are more likely to develop this condition than others. This includes those who are:

• Of Jewish ancestry
• Of Indian, Middle Eastern, or Southeastern European descent
• Are 40 years old and older
• People with autoimmune diseases, especially the disease myasthenia gravis.

Pemphigus Diagnosis

It can be tricky. That’s because a number of conditions can cause blisters. To make sure they find the right cause, your doctor will likely order a number of tests, including:

Medical history. Your doctor will ask you what medications you take, as some drugs can cause this condition. 

A skin exam. Your doctor will use their finger or a cotton swab to rub a patch of your skin that’s not covered by a blister. If it peels easily, you could have pemphigus.

Skin biopsy. Your doctor will take a piece of tissue from one of your blisters and look at it under a microscope.

Blood tests. Your doctor will check your blood for specific antibodies called desmogleins. Antibodies are proteins your body makes to find bad germs and kill them before they harm you.

If you have pemphigus, you’ll have more of these antibodies in your blood than normal. As your symptoms improve, the number of these antibodies in your blood goes down.

Endoscopy. If you have blisters in your mouth, your doctor may use a thin, flexible tube called an endoscope to look down your throat.

Conditions With Similar Symptoms

Some skin diseases can look like pemphigus. Bullous pemphigoid, for example, causes large, fluid-filled blisters, but they generally don’t burst as easily. And unlike pemphigus, it usually affects people over age 60.

Herpes is another condition that can cause painful blisters on your mouth and genitals. But it’s not caused by your body attacking itself.

If you have itchy or painful blisters anywhere on your body, don’t try to figure out what’s wrong. See your doctor. Only they can tell you for sure whether you have pemphigus or something else.

Pemphigus Treatment

Your doctor will likely give you medicine that’ll help ease your symptoms and make you more comfortable. What they prescribe will depend on what type of pemphigus you have and how bad your symptoms are. Treatment might include:

Corticosteroids. These are usually the first line of treatment and can be very effective to relieve symptoms, often within a couple of weeks. They are typically given in pill form.

Immunosuppressants. These drugs keep your immune system from attacking healthy tissues.

Biological therapies. If other medications aren’t working, your doctor may give you a medication called rituximab (Rituxan). You’ll get it in an injection. It helps decrease the number of antibodies that are attacking your body.

Antibiotics, antivirals, and antifungal medications. These fight or prevent infections.

If pemphigus isn’t treated, it could be life-threatening. Sometimes, you may have to be admitted to the hospital until you get better.

Wound care. Topical antibiotics and wound dressings are used to heal the blisters.

Plasmapheresis.  If the pemphigus doesn’t respond to the other treatments, your doctor can use a special machine to take the plasma from your blood. They then replace it with plasma from a blood donor.

Pemphigus Complications

Several serious complications can happen with pemphigus. Among them:

• Skin infections
• Infections that get into the bloodstream (sepsis)
• Being unable to eat, leading to weight loss and malnutrition
• Depression
• Some forms of the condition can cause death if not treated

The medications that you take for pemphigus can also cause serious side effects. They can include:

• Infections
• Rashes
• High blood pressure
• Low bone density (osteoporosis)

At least 75% of people with pemphigus will have a complete remission, or no evidence of the disease, after 10 years of treatment. Some people must take medications for the rest of their lives to keep pemphigus symptoms from coming back.