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Pemphigus

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by Wood D

(Pemphigus Syndromes)

 

Risk Factors

Factors that increase your chance for pemphigus include:
 

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.
Drugs that may be given include the following:
  • 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 with rituximab (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.
 

RESOURCES

American Autoimmune Related Diseases Association, Inc.
http://www.aarda.org

International Pemphigus Foundation
http://www.pemphigus.org

 

CANADIAN RESOURCES

Canadian Dermatology Association
http://www.dermatology.ca

Health Canada
http://www.hc-sc.gc.ca

 

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 Oct;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 Jan 21;(1):CD006263.


Pemphigus. National Institute of Arthritis and Musculoskeletal and Skin Disease (NIAMS) website. Available at: http://www.niams.nih.gov/Health%5FInfo/Pemphigus/default.asp#10. Accessed December 11, 2012.


Pemphigus foliaceus. EBSCO DynaMed website. Available at: https://dynamed.ebscohost.com/about/about-us. Updated May 20, 2011. Accessed December 11, 2012.


Pemphigus vulgaris. EBSCO DynaMed website. Available at: https://dynamed.ebscohost.com/about/about-us. Updated May 20, 2011. Accessed December 11, 2012.

 

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