• Pemphigus

    (Pemphigus Syndromes)

    Definition

    Pemphigus is a group of autoimmune disorders that affects the skin. The attacks cause blisters and burn-like wounds on skin and mucous membranes (like mouth). There are three forms of the disease:
    • Pemphigus vulgaris—most common type of pemphigus
    • Pemphigus foliaceus
    • Paraneoplastic pemphigus—most serious type, usually occurs with cancer

    Causes

    The job of the immune system is to find and attack germs in the body. With pemphigus, the immune system also attacks the 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. For some, medication may be the cause.

    Risk Factors

    Factors that increase your chance for pemphigus include:
    • Family members with pemphigus
    • A history of having autoimmune diseases, such as myasthenia gravis , lupus , or thymoma
    • Jewish or Mediterranean descent
    • Regular use of certain drugs, including:
      • Penicillamine
      • Captopril
      • Rifampin
      • Piroxicam
      • Penicillin
      • 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

    • Lesion may extend deep into the skin
    • Blisters usually start in the mouth or on the scalp
    • Lesions progress to the face, neck, upper body, armpits, and groin
    • Blisters also may occur in the esophagus, rectum, nose, throat, larynx (voice box), eyes, vulva, or rectum
    • Surrounding skin may be red or normal color
    • Pressure on the blister may cause it to expand into surrounding tissue
    • Outer layer of skin can easily be rubbed off
    • Blisters are soft and break easily, releasing fluid
    • Open sores are painful
    • Large areas of skin may open up, increasing the risk of fluid imbalance and infection
    • Blisters usually heal without scarring, but skin color may change to brown

    Pemphigus Foliaceus

    • Lesions are superficial
    • Blisters are itchy or produce a burning sensation
    • Sores are usually not found in the mouth or on other mucus membranes
    • Blisters usually first show up on the face, scalp, chest, or upper back
    • Blisters open, causing shallow sores
    • Skin is red
    • Scales and crusts form
    • Sunlight may make symptoms worse

    Paraneoplastic Pemphigus

    • Sores occur on the mucous membranes, in the mouth, eye, and esophagus
    • Blisters often appear on palms of hands and soles of feet
    • Lesions are itchy or painful

    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
    Skin proceedure
    Copyright © Nucleus Medical Media, Inc.

    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
    • Medicated mouthwash—for pain relief
    • IV immunoglobulin—immune system elements from a healthy immune system, often given in combination with rituximab
    • 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 can not 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. 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, Daneshpazhooh M, Valikhani M, Balighi K, Hallaji Z, Barzegari M, Akhyani M, Ghodsi SZ, Seirafi H, Nazemi MJ, Mortazavi H, Mirshams-Shahshahani M. Randomized controlled open-label trial of four treatment regimens for pemphigus vulgaris. J Am Acad Dermatol . 2007 Oct;57(4):622-8.

    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, Villanueva E, Segall J, Murrell DF. 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: http://www.ebscohost.com/dynamed/what.php . Updated May 20, 2011. Accessed December 11, 2012.

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

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