PEMPHIGUS: OVERVIEW
Pemphigus vulgaris: Without treatment, the blisters and painful sores can become widespread, as shown here.
What is pemphigus?
Pemphigus (pem(p)-fi-gəs) is a group of rare diseases that causes blisters. There are many different types of pemphigus, including:
- Pemphigus vulgaris (vul-gar-is)
- Pemphigus foliaceus (foe-lay-s(h)e-əs)
- Drug-induced pemphigus
- Fogo selvagem (foe-go sell-va-gem)
- Paraneoplastic pemphigus
Regardless of type, the blisters are soft, limp, and break open easily.
Most people get blisters on their skin. With some types of pemphigus, blisters form inside the mouth. A few types cause blisters on moist tissues like those that line the inside of the nose and eyes, throat, anus, or genitals.
Anywhere the blisters form, they tend to break open quickly, leaving painful sores. In the throat, the sores can cause hoarseness. Mouth sores can make eating, drinking, and talking difficult.
When sores develop, they tend to heal slowly. Some may never heal.
Pemphigus can develop in otherwise healthy people.
The important thing to remember is that today pemphigus in nearly always controllable. By keeping your dermatology appointments, you can partner with your dermatologist to make that a reality.
PEMPHIGUS: SIGNS AND SYMPTOMS
When someone develops pemphigus, it can develop slowly, causing blisters in the same area for years.
Signs and symptoms can also show up quickly. Blisters can appear suddenly and spread. Widespread pemphigus can be life threatening. It can turn an otherwise healthy person into one who is extremely sick, incredibly tired, and in pain.
The following explains how pemphigus affects different areas of the body.
Skin
On the skin, blisters usually begin in one area. They can develop on normal-looking skin or skin that looks inflamed. Either way, the blisters soon break open and ooze fluids. They then become sores partly covered with crust.
The following pictures show what pemphigus can look like on the skin.
Drug-induced pemphigus: Some people get pemphigus after taking certain medicines. This man took penicillamine, which is the most common cause of drug-induced pemphigus.
Pemphigus vulgaris: The blisters have burst, leaving painful sores. About 70% of people who get pemphigus have this type.
Pemphigus foliaceus: This type of pemphigus affects only the skin and often develops on the face, scalp, and upper body.
The sores are often painful, but rarely itchy. Some people say the affected skin burns.
The sores tend to heal slowly, and some never heal. When a sore heals, you may see a dark spot in its place. This is not a scar. Some people see dark spots when their skin heals. The dark spots often fade on their own, but this can take time.
Mouth sores: Most people who have pemphigus vulgaris develop mouth sores at some point.
Mouth and throat
Painful mouth sores are common in people who have pemphigus vulgaris, the most common type of pemphigus. About 50% to 70% of people who have pemphigus vulgaris develop mouth sores before blisters appear on their skin.
Mouth sores begin as blisters, which quickly burst causing the painful sores.
These sores can be so painful that some people stop eating solid food and use a straw to drink. If sores develop in the throat, talking can be painful.
Some people see the blisters spread from their mouth to their lips and then their skin.
Nail problems: While rare, pemphigus can affect the nails and surrounding skin.
Nails
Nail problems develop in some people who have severe pemphigus. An infection may develop in the skin around the nail as shown here. Some people see their nails slowly disappear.
With treatment, most people can recover lost nails.
Moist tissues
Painful sores can develop in the tissue lining the inside of the eyes and nose, genitals, anus, and other areas of the body. Even the esophagus (the tube that connects the throat to the stomach) can develop blisters and sores in rare cases.
Other signs and symptoms of pemphigus
- Pain
- Fatigue
- Weakness
- Light sensitivity
- Eye problems
If you notice blisters that suddenly appear on your skin, inside your mouth, or elsewhere, immediately make an appointment to see a dermatologist. Many skin diseases can cause blisters. An accurate diagnosis is essential.
You’ll find pemphigus in countries around the world and in people of all races. About the same number of men and women get it.
Most types of pemphigus are rare in children. Middle-aged or older people are most likely to get pemphigus, including the most common type, pemphigus vulgaris. This type usually begins between 50 and 60 years of age.
Your risk of getting pemphigus vulgaris increases if you have:
- Jewish ancestry, especially Ashkenazi Jewish heritage
- Mediterranean ancestry
- An autoimmune disease, especially myasthenia gravis (my-as-thē-knee-ah grav-is)
The risk of getting other types of pemphigus increases if you:
Take certain medicines: People who take penicillamine (pen-nə-cil-la-mean) have a higher risk of getting pemphigus foliaceus (foe-lay-s(h)e-əs) or pemphigus vulgaris. People take this medicine to treat rheumatoid arthritis and Wilson’s disease. It’s also used to prevent kidney stones.
Some other medicines can also increase the risk of getting pemphigus. When a drug causes pemphigus, the person has drug-induced pemphigus.
Live in a rural, tropical area of Brazil or another country in Latin America: People in these areas have a higher risk of getting a type of pemphigus called fogo selvagem, which only occurs in these areas. Children and young adults are most likely to get fogo selvagem.
Researchers have found that when these areas become less rural, fewer people get fogo selvagem. When a rural area becomes a city, people no longer get this type of pemphigus.
Have a tumor growing inside your body, especially if it starts in one of these areas — lymph node, tonsil, spleen, or thymus gland: The tumor can be benign (non-cancerous), a pre-cancer, or cancer. Some people who have a tumor also get paraneoplastic pemphigus. This is the rarest type of pemphigus.
What causes pemphigus?
People get pemphigus when their body’s immune system attacks healthy cells in their:
- Skin
- Mouth, throat, or both
- Moist tissues that lines the inside of the nose, eyelids, anus, genitals, or other areas
The body attacks these healthy cells because it mistakes them for something harmful like viruses or bacteria.
Scientists are still trying to find out why the body does this. We know that pemphigus is NOT contagious, so it’s not something you catch that causes these attacks.
Finding what causes pemphigus could lead to safer and more effective treatments.
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:
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- 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.
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.
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 2 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.