Pemphigus
En Español (Spanish Version)

Definition
Pemphigus is a group of disorders that affects the skin. The attacks cause blisters and burn-like wounds on skin and mucous membranes (like mouth). There are 3 forms of the disease:

  • Pemphigus vulgaris—most common type of pemphigus
  • Pemphigus foliaceus
  • Paraneoplastic pemphigus—most serious type, usually occurs with cancer
Causes
Pemphigus is an autoimmune disorder. The immune system attacks healthy skin and mucus membranes. The attack causes the sores on the skin.

It is not clear what causes the immune system to attack normal body tissue. It is likely due to a combination of genetic and environmental factors. For some, medication may be the cause.

Risk Factors
Factors that may increase your chance of pemphigus include:

  • Family members with pemphigus
  • A history of having autoimmune diseases, such as myasthenia gravis, systemic lupus erythematosus, or thymoma
  • Jewish or Mediterranean descent
  • Regular use of certain medications:
    • Chelating agents, such as penicillamine
    • ACE inhibitors
    • Nonsteroidal anti-inflammatory drugs (NSAIDs), such as aspirin
    • Antibiotics, such as penicillin
    • Antiseizures, such as phenobarbital
Symptoms
Pemphigus may occur over a small or large section of the skin. Itching and pain are common symptoms. Other symptoms will vary according to the type of pemphigus:

Pemphigus vulgaris may cause:

  • Lesion that may extend deep into the skin
  • Blisters that usually start in the mouth or on the scalp
  • Lesions that progress to the face, neck, upper body, armpits, and groin
  • Blisters which may appear in the esophagus, rectum, nose, throat, larynx (voice box), eyes, vulva, or rectum
  • Red skin
  • Painful, open sores
  • Blisters that may expand into surrounding tissue when pressure is added to them
  • An outer layer of skin to be easily rubbed off
  • Soft and easily broken blisters that release fluid
  • Large areas of open skin, increasing the risk of fluid imbalance and infection
  • Blisters that usually heal without scarring, but may change skin color to brown
Pemphigus foliaceus may cause:

  • Superficial lesions
  • Blisters that itch or produce a burning sensation
  • Sores that are usually not found in the mouth or on other mucus membranes
  • Blisters that first show up on the face, scalp, chest, or upper back
  • Open blisters, causing shallow sores
  • Red skin
  • Scales and crusts
  • Worsening symptoms in sunlight
Paraneoplastic pemphigus may cause:

  • Sores on mucous membranes, in the mouth, eye, and esophagus
  • Blisters that appear on palms of hands and soles of feet
  • Itchy or painful lesions
Diagnosis
The doctor will ask about your symptoms and medical history. A physical exam will be done. This will include a thorough skin exam. Special care is given to examining the lesions.

Tests to look for signs of an autoimmune disorder may include:

  • Skin biopsies of the lesion and surrounding tissue
  • Blood and skin tests
Skin Biopsy

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Treatment
There is no cure for pemphigus but treatment may help control your condition. If left untreated, pemphigus can lead to death.

Treatment aims to control the disease and prevent infection of the blistering lesions.

If a drug triggered pemphigus, the doctor will stop that medication. In some cases, stopping the drug is all that is needed for recovery.

Wound Care
Wound care is important to prevent infections. The wounds are treated similarly to severe burns. They will be cleaned and protected from further contamination. Antibiotics medication will also be applied to the wounds to prevent infections.

Medication
Medications can usually help control symptoms. However, these medications can also produce serious side effects. Your doctor may order blood and urine tests to check for adverse effects.

Medications to help control pemphigus include:

  • Steroids—reduce swelling and calm the immune system
  • Immunosuppressive medications—decrease the strength of your immune system
  • Antibiotics—treat or prevent infection
  • Medicated mouthwash—for pain relief
  • IV immunoglobulin—immune system elements from a healthy immune system, often given in combination a biologic antineoplastic agent
  • Topical steroids, including injection into specific lesions
It takes some time for the immune system to clear. In fact, it may be several months or years for the skin sores to disappear. Some may have a complete remission after a period of medication treatment. Others will need to continue smaller doses of medication between outbreaks.

Mouth Care
Sores in the mouth can make eating and dental care difficult. It is important to maintain good dental hygiene. If you cannot brush because of mouth sores, talk to your dentist. They can provide alternative ways to keep you mouth clean.

Lesions in the mouth can also make it difficult to eat. Soft diets may help until the lesions heal. Talk to a dietitian if you are having trouble getting adequate nutrition with a soft diet.

Avoid foods that can cause irritation in your mouth. This includes foods that are high in acids, spicy, or hard.

Lifestyle Changes
Good overall nutrition helps the body heal and fight disease.

Some foods may also make your symptoms worse. They may also trigger the onset of more sores. Foods that have been linked to these problems include garlic, onions, and leeks. Keep track of the foods that seem to cause a reaction in your skin. Read the labels of all foods to make sure they do not contain foods that can set off a reaction.

Surgery
Paraneoplastic pemphigus may require the removal of a tumor. The removal may improve the disorder or decrease symptoms.

Prevention
There are no guidelines for preventing pemphigus because the cause is unknown.




RESOURCES:
American Autoimmune Related Diseases Association, Inc.

International Pemphigus Foundation

CANADIAN RESOURCE:
Canadian Dermatology Association


References:
Beers MH, et al. The Merck Manual of Medical Information—Home Edition. 2nd ed. Simon and Schuster, Inc.; 2003.

Chams-Davatchi C, Esmaili N, et al. Randomized controlled open-label trial of four treatment regimens for pemphigus vulgaris. J Am Acad Dermatol. 2007;57(4):622-628.

Ferri FF. Ferri's Clinical Advisor: Instant Diagnosis and Treatment. 8th ed., St. Louis: Mosby; 2006.

Goldman L. Cecil Textbook of Medicine. 22nd ed. Philadelphia: Saunders; 2004.

Griffith's 5-Minute Clinical Consult. 2001 ed. Lippincott Williams & Wilkins; 2001.

Martin LK, Werth V, et al. Interventions for pemphigus vulgaris and pemphigus foliaceus. Cochrane Database Syst Rev. 2009;(1):CD006263.

Pemphigus. National Institute of Arthritis and Musculoskeletal and Skin Disease (NIAMS) website. Available at: http://www.niams.nih.gov/Health_Info/Pemphigus/default.asp#10. Accessed December 11, 2012.

Pemphigus foliaceus. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed. Updated May 20, 2011. Accessed December 11, 2012.

Pemphigus vulgaris. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynameds. Updated May 20, 2011. Accessed December 11, 2012.

Last Reviewed November 2014



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