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Pemphigus by Debra Wood, RN

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

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 RESOURCE:

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_Info/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.

Last reviewed November 2012 by Michael Woods, MD

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