Pemphigus vulgaris

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Pemphigus, vulgaris - lesions in the mouth

Pemphigus, vulgaris on the back

Severe cases of pemphigus may need wound management, similar to the treatment for severe burns. People with this condition may need to stay in a hospital and receive care in a burn unit or intensive care unit.

Treatment is aimed at reducing symptoms, including pain. It also aims to prevent complications, especially infections.

Treatment may involve:

• Antibiotics and antifungal medications to control or prevent infections
• Fluids and electrolytes given through a vein (IV)
• IV feedings if there are severe mouth ulcers
• Numbing (anesthetic) mouth lozenges to reduce mouth ulcer pain
• Pain medications if local pain relief is not enough

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Body-wide (systemic) therapy is needed to control pemphigus and should be started as early as possible. Systemic treatment includes:

• An anti-inflammatory drug called dapsone
• Corticosteroids
• Medications containing gold
• Medications that suppress the immune system (such as azathioprine, methotrexate, cyclosporin, cyclophosphamide, mycophenolate mofetil, or rituximab)

However, side effects from systemic therapy are a major complication.

Some antibiotics are also effective, particularly minocycline and doxycycline. Intravenous immunoglobulin (IVIg) is occasionally used.

Plasmapheresis is a process in which antibody-containing plasma is removed from the blood and replaced with intravenous fluids or donated plasma. Plasmapheresis may be used along with systemic medications to reduce the amount of antibodies in the blood.

Ulcer and blister treatments include soothing or drying lotions, wet dressings, or similar measures.

Without treatment, this condition is usually life-threatening. Severe infection is the most frequent cause of death.

With treatment, the disorder tends to be chronic. Side effects of treatment may be severe or disabling.

• Secondary skin infections
• Severe dehydration
• Side effects of medications
• Spread of infection through the bloodstream (sepsis)

Your health care provider should examine any unexplained blisters.

Call your health care provider if you have been treated for pemphigus vulgaris and you develop any of the following symptoms:

• Chills
• Fever
• General ill feeling
• Joint aches
• Muscle aches
• New blisters or ulcers

Review Date: 09/11/2010
Reviewed By: Linda J. Vorvick, MD, Medical Director, MEDEX Northwest Division of Physician Assistant Studies, University of Washington, School of Medicine; and Roy Colven, MD, Dermatologist, Associate Professor of Medicine, University of Washington Medical School, Seattle, Washington. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org).