Pemphigus

  • Overview
    pemphigus-vulgaris.jpg
    Pemphigus vulgaris: Without treatment, the blisters and painful sores can become widespread, as shown here.

    Pemphigus: Overview

    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.

    Image used with permission of the American Academy of Dermatology National Library of Dermatologic Teaching Slides.


    References
    James WD, Berger TG, et al. “Chronic blistering dermatoses.” In: James WD, Berger TG, et al. Andrews’ Diseases of the Skin (10th edition). Saunders Elsevier, Philadelphia, 2006:459-66.

    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.

    Zeina B. (DM Elston, editor). Pemphigus vulgaris. Medscape. Updated 8.12.2016. Last accessed 8.23.2016.


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  • Symptoms

    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.

    pemphigus-vulgaris-skin.jpg   pemphigus-foliaceus-skin.jpg
    drug-induced-pemphigus-skin.jpg
    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.
      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.

     

    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 and throat

      pemphigus-vulgaris-mouth.jpg
    Mouth sores: Most people who have pemphigus vulgaris develop mouth sores at some point.

    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.

    Nails

    pemphigus-vulgaris-nails.jpg
    Nail problems: While rare, pemphigus can affect the nails and surrounding skin.

    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.

    Images 1 – 4 used with permission of the American Academy of Dermatology National Library of Dermatologic Teaching Slides.

    Image 5: (J Am Acad Dermatol 2000;43:529–35.)


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

    Habif TP, Campbell, JL, et al. “Vesicular and bullous diseases.” In: Dermatology DDxDeck. Mosby Elsevier, China, 2006: Cards# 100 and 101.

    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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  • Causes

    Pemphigus: Who gets and causes

    who-gets-pemphigus.jpg
    Although pemphigus is rare, it develops in people around the world.

    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.


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

    Habif TP, Campbell, JL, et al. “Vesicular and bullous diseases.” In: Dermatology DDxDeck. Mosby Elsevier, China, 2006: Cards# 100 and 101.

    James WD, Berger TG, et al. “Chronic blistering dermatoses.” In: James WD, Berger TG, et al. Andrews’ Diseases of the Skin (10th edition). Saunders Elsevier, Philadelphia, 2006:459-66.


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  • Treatment

    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.

    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.

    pemphigus-vulgaris-treatment.jpg
     

    • 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.

    Taking both a corticosteroid like prednisone and a medicine like azathioprine to quiet the immune system may deliver better results.

    pemphigus-vulgaris-treatment2.jpg
     

    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.


    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.


    Pemphigus

  • Tips

    Pemphigus: 10 tips for managing

    Pemphigus can be a serious disease that changes your life. The following can help you manage life with pemphigus.

    1. Try NOT to injure your skin. If you cut, bump, or injure your skin in any way, new blisters can form.

    2. Take precautions to avoid infections. Blisters and sores leave you more likely to develop an infection. Many medicines taken to control the pemphigus also increase your risk of getting an infection because the medicines suppress your immune system.

      To decrease your risk of getting an infection, keep all wounds, including cuts and scrapes, clean.

    If you are unsure how to take care of your blisters and sores, ask your dermatologist.

    pemphigus-vulgaris-tips.jpg
     

    1. Understand that it can take time to get pemphigus under control. Some patients need months or years, but pemphigus is nearly always controllable.

    2. Try to remain calm. Learning that you have pemphigus can be very stressful as can living with the symptoms. Stress, however, can worsen pemphigus. Finding things that help you combat stress can be immensely helpful.

    3. Take care of mouth sores. If pemphigus causes sores in your mouth, you can ease the pain by:

    • Eating bland, soft foods. You want to avoid hard foods like chips, chunky peanut butter, nuts, crisp vegetables like raw carrots, and fruit. Other foods that can cause new mouth sores include spicy foods, steaming-hot foods, and acidic foods like tomatoes and citrus fruits.

    • Drinking beverages that don’t cause pain. Orange juice, hot tea and coffee, and spicy drinks can irritate your mouth, causing new sores.

    • Keeping your mouth clean. Poor oral hygiene can worsen mouth sores. Your dermatologist can recommend a toothbrush, toothpaste, and mouthwash that are least likely to cause pain.

    • Seeing a dentist who has experience working on patients with pemphigus. A dentist with this experience can take precautions to avoid new blisters.

    • Asking your dermatologist if a painkiller or anesthetic could help ease the mouth pain while you get pemphigus under control. Some people find these medications allow them to brush their teeth and eat.

    1. Tell your dermatologist about eye problems. Blisters can form on the tissue lining an eyelid. Some people get pink eye with lots of mucus or crusts along their eyes. For a while, your eyes may become very sensitive to light.

      If pemphigus affects your eyes, you may need to:

    • Wear glasses instead of contact lenses.
    • Wear sunglasses that protect your eyes from different types of light and ease light sensitivity.
    • Follow a treatment plan for your eyes.

    1. Avoid sun and heat if you have pemphigus foliaceus or drug-induced pemphigus. If you’ve been diagnosed with pemphigus foliaceus or one of its subtypes, such as drug-induced pemphigus or fogo selvagem, you want to:

    • Avoid being out in the sun
    • Stay away from heat

    These can cause the blisters to spread, so you get blisters where you previously had none.

    1. Learn more about pemphigus. The following websites can help you get started:

      International Pemphigus and Pemphigoid Foundation
      Provides hope and a new perspective for people living with pemphigus by offering patient support, personal stories, news, and more.

      There’s definitely life after pemphigus
      This woman’s story can give you hope.

    2. Connect with others who have pemphigus. People often find inspiration and hope by sharing experiences and connecting with others who have pemphigus. The following links allow you to ask a health coach who has pemphigus a question or meet others who have pemphigus:

      Ask a coach
      Pemphigus and pemphigoid community

    3. Partner with your dermatologist. Your dermatologist can be your strongest ally. Dermatologists help patients with pemphigus find treatment that works. No one treatment works for everyone.

      Your dermatologist can also help you manage possible side effects and answer questions about the disease.

    If you remember only one thing from this site, it should be that today pemphigus is nearly always controllable. By keeping your dermatology appointments, you can partner with your dermatologist to make that a reality.


    References
    The Marshall Protocol Knowledge Base, “Photosensitivity.” Last accessed 8.23.2016.

    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.

    Zeina B. (DM Elston, editor). Pemphigus vulgaris. Medscape. Updated 8.12.2016. Last accessed 8.23.2016.


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