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Pemphigus: Diagnosis and treatment


How do dermatologists diagnose pemphigus?

Dermatologists are the experts in skin disease, so they are often asked by other doctors to examine a patient who could have pemphigus. Many different skin diseases cause blistering, and an accurate diagnosis is so important.

If a dermatologist suspects you may have pemphigus, you’ll likely need the following:

  • Physical exam: Your dermatologist will look at the blisters, sores, and crusts. If you have blisters on your skin, your dermatologist will lightly rub a bit of normal-looking skin near a blister to find out if the skin comes off.

  • Medical history: Your dermatologist will ask questions about your health and what medicines you take.

  • Skin biopsy: Your dermatologist can easily perform a skin biopsy during the office visit. To do this, your dermatologist will remove part of a new blister and some normal-looking skin (or other tissue) from near the blister. These will be looked at under a microscope in a lab.

  • Blood test: This test can tell whether you have proteins in your blood that cause pemphigus.

If you are diagnosed with pemphigus, you'll likely need treatment. Without treatment, pemphigus tends to worsen. In some people, pemphigus can become life-threatening without treatment.

Some medicines can cause or worsen pemphigus

Be sure your dermatologist has a list of all the medicines you take, including ones you buy without a prescription.

How do dermatologists treat pemphigus?

Pemphigus cannot be cured, but with treatment, most people can control their pemphigus.

Treatment can reduce (and sometimes clear) the blisters and sores caused by all types of pemphigus. Treatment can also prevent pemphigus from worsening.

A treatment plan for pemphigus may include one or more of the following:

Corticosteroid: If you have mild pemphigus, your dermatologist may prescribe a corticosteroid that you apply to your skin. Many people need stronger medicine like prednisone or methylprednisolone. These corticosteroids work throughout the body.

A corticosteroid can clear the blisters and sores.

Immunosuppressant medication: This medication quiets (or suppresses) the immune system. Either azathioprine or mycophenalate mofetil is often used to treat pemphigus. These can stop the body from creating new blisters.

Biologics: This is a newer treatment option. One biologic, rituximab, seems promising. It appears to offer safe treatment. Because pemphigus is rare, we don’t have the studies needed to know who will respond and what long-term side effects are possible.

Antibiotics, antivirals, and antifungals: If you have an infection, this type of medicine will be part of your treatment plan.

Wound care: Your dermatologist may include baths and wound dressings in your treatment plan. This can help heal blisters and sores.

Other medicines: These can be helpful for many reasons. For some people, prednisone and the other medicines described above fail to control the pemphigus. Another medicine may work well. Switching to another medicine can also help prevent possible side effects that can occur when someone takes a corticosteroid for a long time.

Combined treatments

Taking both a corticosteroid like prednisone, and an immunosuppresant medication like azathioprine to quiet the immune system, may deliver better results.

When pemphigus is severe or medicine fails to work, a dermatologist may talk with you about one of the following treatment options

Plasmapheresis: This treatment involves removing plasma from your blood. Plasma contains the proteins that cause your immune system to attack your skin and the moist tissues lining your mouth and other parts of your body.

During plasmapheresis, a machine filters the plasma from your blood. Your own plasma is replaced with healthy plasma from a blood donor and then returned to you.

Extracorpeal photochemotherapy: This treatment begins with a blood draw. After your white blood cells are removed from the drawn blood, the rest of the blood is returned to your body. The white blood cells are treated with a medicine called psoralen and then exposed to UVA light. This kills the diseased white blood cells. The treated blood is then returned to your body.

This treatment usually takes two days, but you can go home each day after treatment.

Hospital stay: To treat health problems that pemphigus can cause, some patients need to be hospitalized. While in the hospital, a patient may get an IV to replace lost fluids. Widespread sores can cause an enormous loss of fluids. An IV can also help patients get much-needed nutrition. Sores in the mouth or throat can make it too painful to eat.

While in the hospital, treatment can also be given to help get pemphigus under control.

Researchers are looking for better treatment options

To give patients safer and more effective treatment options, researchers continue to study pemphigus.

One area of research shows promise. At the University of Pennsylvania School of Medicine, scientists have found a way to successfully remove the cells that cause pemphigus vulgaris. They have successfully treated mice using this technique, without harming healthy tissue.

This could lead to better treatment for pemphigus vulgaris.

What is the outcome for someone with pemphigus?

Today, the outcome looks good. For most people, the disease can be controlled with treatment. Many can eventually stop their treatment for a while.

Before medicines like prednisone and azathioprine were used to treat pemphigus, a person lived about 5 years after getting pemphigus vulgaris, the most common type. Thanks to medicines and other treatments, this has changed. Few people die of pemphigus.

People still have to deal with other problems that pemphigus can cause. The medicines can cause serious side effects.

Other problems include taking the time to see doctors and get treatment. To keep pemphigus under control, you may need to see both a dermatologist and your primary care physician. If pemphigus affects your eyes, you’ll also need to see an eye doctor. You may also see a dentist for help with pemphigus or a doctor who specializes in the ears, nose, and throat.

Serious weight loss is another problem that pemphigus can cause.

These problems can be managed with help from your dermatologist and other doctors.


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References
Amagai M. “Pemphigus.” In: Bolognia JL, et al. Dermatology. (second edition). Mosby Elsevier, Spain, 2008:417-29.

Craythorne EE Mufti G, et al. “Rituximab used as a first-line single agent in the treatment of pemphigus vulgaris.” J Am Acad Dermatol. 2011 Nov;65(5):1064-5.

Huang A, Madan RK et al. “Future therapies for pemphigus vulgaris: rituximab and beyond.” J Am Acad Dermatol. 2016 Apr;74(4):746-53.

National Institutes of Health. “Questions and answers about pemphigus.” Last updated June 2015. Last accessed August 18, 2016.

News Medical. “Two new studies find potential genetic cause and new treatment method for autoimmune diseases.” July 13, 2016. Last accessed August 16, 2017.

Stanley JR. “Pemphigus.” In: Wolff K, Goldsmith LA, et al. Fitzpatrick’s Dermatology in General Medicine (seventh edition). McGraw Hill Medical, New York, 2008: 459-74.

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