Pemphigus

About

Pemphigus

What is pemphigus?

Pemphigus is a group of rare diseases that cause blisters and sores throughout the body on mucous membranes and skin. It usually happens to middle-aged or older people, especially those of Jewish or Mediterranean heritage. This chronic (long term) condition has no cure but can be controlled with medication. There are several types, and the most severe can be life-threatening if not treated. It is not contagious.

Why should I worry about pemphigus?

Although pemphigus is a rare disease, if you get it, it can cause serious complications including:

  • Skin infections
  • Sepsis, an Infection that spreads to your bloodstream, and can be life-threatening
  • Malnutrition because it’s painful to eat with mouth sores
  • Side effects from medication such a high blood pressure
  • Death, if certain types are not treated

Causes

What causes pemphigus?

Pemphigus is a rare autoimmune disease. Your immune system keeps you healthy by attacking germs, viruses, bacteria and other organisms that invade your body. For an unknown reason, an autoimmune disease produces antibodies that cause the immune system to attack the body. Pemphigus produces antibodies that damage the body’s skin and mucous membranes in the mouth, throat, nose, eyelids, anus, genitals and other areas. It can also be caused by a reaction to taking certain medications, chiefly penicillamine.

Who’s at risk for pemphigus?

Your risk of getting pemphigus is higher if you:

  • Are of Jewish heritage, especially Ashkenazi Jew
  • Have Middle Eastern or Mediterranean ancestry
  • Have another autoimmune disease, especially myasthenia gravis
  • Take certain medications to treat rheumatoid arthritis, Wilson’s disease or to prevent kidney stones
  • Live countries where pemphigus is common in rural areas

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Initial Visit

$75

Follow Up

Symptoms

What are the symptoms of pemphigus? 

Pemphigus can develop slowly, or appear suddenly and spread quickly. An otherwise healthy person can quickly become very sick, with pain and extreme fatigue. Widespread blisters can be life threatening.

Pemphigus symptoms depend on the type you have. Many cases can cause these symptoms:

  • Blisters on skin and mucous membranes that are soft and limp. Blisters begin in one area, on both normal-looking skin or inflamed skin.
  • Blisters break open easily, ooze fluid and become open sores; sores can crust over.
  • Sores can develop in the mouth, eyes, nose, genitals, anus, throat or esophagus.
  • Sores can become widespread, infected and painful.
  • Sores in the mouth or throat can cause hoarseness, make talking difficult, and cause painful eating and drinking.
  • Sores heal very slowly or may never heal, leaving blisters in the same area for many years.
  • Infections can develop in the skin around fingernails, causing the nail to slowly disappear. Treatment can recover lost nails.
  • Extreme fatigue, or weakness and pain.
  • Sensitivity to light and eye problems.
  • Severe weight loss.

Are there different types of pemphigus?

Yes, there are many types including:

  • Pemphigus vulgaris is the most common type, causing up to 70% of all pemphigus. It’s most common in people between the ages of 30 and 60. Blisters begin in the mouth that quickly burst and leave painful sores that make eating extremely painful. Later, blisters spread to the lips and then develop on the skin.
  • Pemphigus foliaceus only occurs on the skin, with blisters developing on the face, scalp, chest, back and shoulders. The blisters are not painful but can be very itchy and form a crust.
  • Pemphigus erythematosus causes blisters on your scalp, cheeks, upper back and chest that become red with crusty lesions. 
  • Drug-induced pemphigus is most often caused by the drug penicillamine. It causes painful sores that burn and heal slowly, leaving a dark spot after the sore heals. The blisters can form up to six months after you’ve taken the drug. 
  • Pemphigus vegetans is similar to pemphigus vulgaris, except thick, wart-like lesions develop instead of blisters. They usually develop where skin rubs against skin like armpits or groin area. 
  • Endemic pemphigus or fogo selvagem is rare, occurring most often in rural areas of Brazil or South and Latin American countries. It often affects more than one member of a family, and can develop in children and young adults.
  • Paraneoplastic pemphigus is the rarest type and is caused by a cancerous tumor in a lymph node, tonsil, spleen or thymus gland. It causes blisters in the mouth. Treating the cancer can help ease the blisters.

Diagnosis

How is pemphigus diagnosed?

Your doctors may refer you to a dermatologist (medical doctors who specialize in skin conditions) because pemphigus is difficult to diagnose. Many conditions and diseases can cause blisters so an accurate diagnosis is essential to help direct treatment. Be sure your dermatologist has a list of all medications you take, including over-the-counter drugs.

In addition to a physical exam of your skin and mouth, you may need tests, including:

  • Skin biopsy removes a piece of tissue from a blister and it’s examined under a microscope.
  • Blood tests can identify antibodies that are present with pemphigus.
  • Endoscopy can check for sores in your throat, using a flexible, lighted tube.

Treatment

What are the treatment options for pemphigus?

Without treatment, pemphigus tends to worsen. Some types of pemphigus can become life-threatening without treatment. Treatment usually begins with medications to stop more blisters from forming. The earlier treatment starts, the more effective it will be. If a medication triggers your pemphigus, stopping that drug may be sufficient to stop your pemphigus. Many people improve slowly with medications. Others may need to take medication for the rest of their life to keep symptoms from returning. 

Medications depend on the type and severity of your disease, and if you have other health conditions. Before medicines like prednisone were used to treat pemphigus vulgaris, patients only lived about five years. Today, very few people die of pemphigus.  

Treatment may include these medications, prescribed alone or in combination:

  • Corticosteroid cream may bring relief in mild cases. More serious cases require an oral corticosteroid such as prednisone pills. Taking corticosteroids for a long time (or in high doses) can cause serious side effects. They can include diabetes, bone loss, higher chance of infections, stomach ulcers and redistribution of body fat to the face (moon face).
  • Immunosuppressant drugs can keep your immune system from attacking the body’s healthy tissues, and can stop the body from creating new blisters. However, these drugs can increase your risk of infections. 
  • Other medications may be required if the above are not helping. Your dermatologist may prescribe dapsone, intravenous immunoglobulin or rituximab. 
  • If you have an infection, you may need antibiotics, antivirals, or antifungals.
  • Biologics are showing promise against pemphigus.

Very serious cases, or if treatment isn’t working, may benefit from one of these treatments:

  • Plasmapheresis removes plasma from your blood, and it’s replaced with healthy plasma from a blood donor. The proteins that cause your immune system to attack skin and mucous membranes are contained in blood plasma. 
  • Extracorporeal photochemotherapy removes white blood cells from your blood. They are treated with a medication, and exposed to ultraviolet-A light to kill the diseased white cells. The treated blood is returned to your body.
  • Hospitalization may be needed to get an intravenous (IV) to replace the large amount of fluid lost from widespread sores, and to get much-needed nutrition. Medications can be started in the hospital to get pemphigus under control. 

Lifestyle changes and home remedies should be included in treatment plans. 

  • Follow your doctor’s wound-care instructions to prevent infections and scars. Use mild soap to clean your skin and apply moisturizer immediately afterward.
  • Avoid activities where you could hurt your skin, because even a minor injury can cause new blisters.
  • Avoid spicy, hot or rough foods if you have active blisters in your mouth. 
  • Stay out of the sun as much as you can because ultraviolet light can trigger new blisters. High temperatures can also worsen your symptoms and cause blisters to spread. 
  • If it’s difficult to brush your teeth because of mouth blisters, ask your dentist how to clean your mouth, teeth and gums without injuring them. Ask about an anesthetic to reduce the pain of eating and brushing your teeth.
  • Pemphigus can be very difficult to live with and you may benefit from a support group, and regular stress reduction techniques or activities.
  • If your eyes are involved, avoid contact lenses and wear sunglasses to ease light sensitivity.

References

Mayo Clinic. (Oct. 2020). Pemphigus. Retrieved 9-27-21, {https://www.mayoclinic.org/diseases-conditions/pemphigus/symptoms-causes/syc-20350404}
American Academy of Dermatology Association. (Aug. 2016). Pemphigus: Overview. Retrieved 9-27-21, {https://www.aad.org/public/diseases/a-z/pemphigus-overview}
American Academy of Dermatology Association. (Aug. 2016). Pemphigus: Signs and Symptoms. Retrieved 9-27-21, {https://www.aad.org/public/diseases/a-z/pemphigus-symptoms}
American Academy of Dermatology Association. (Aug. 2016). Pemphigus: Who Gets and Causes. Retrieved 9-27-21, {https://www.aad.org/public/diseases/a-z/pemphigus-causes)
American Academy of Dermatology Association. (Aug. 2016). Pemphigus: Diagnosis and Treatment. Retrieved 9-27-21, {https://www.aad.org/public/diseases/a-z/pemphigus-treatment}
American Academy of Dermatology Association. (Aug. 2016). Pemphigus: 10 Tips for Managing. Retrieved 9-27-21, {https://www.aad.org/public/diseases/a-z/pemphigus-self-care}
WebMD. (July 2020). Pemphigus. Retrieved 9-27-21, ({https://www.webmd.com/skin-problems-and-treatments/what-is-pemphigus}

Information

Medically reviewed by:

Dr. Javeed Siddiqui, MD, MPH

Dr. Siddiqui is the Chief Medical Officer at TeleMed2U responsible for clinical and technical program development as well as maintaining a thriving telemedicine practice in infectious diseases which includes specialized care of Hepatitis and HIV.

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