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Pemphigus vulgaris

• Overview
• Symptoms
• Treatment

Treatment:

Severe cases of pemphigus are treated similarly to severe burns . Treatment may require hospitalization, including care in a burn unit or intensive care unit. Treatment is aimed at reducing symptoms and preventing complications.

Intravenous fluids, electrolytes , and proteins may be required. Mouth ulcers , if severe, may mean intravenous feeding is needed. Anesthetic mouth lozenges may reduce the pain of mild to moderate mouth ulcers. Antibiotics and antifungal medications may be appropriate to control or prevent infections.

Systemic therapy as early as possible is required to control pemphigus, but side effects from systemic therapy are a major complication. Treatment includes corticosteroids, medications containing gold, the anti-inflammatory drug dapsone, or medications that suppress the immune system (such as azathioprine, methotrexate, cyclosporin, cyclophosphamide, or mycophenolate mofetil). Some antibiotics are also effective, particularly minocycline and doxycycline. Intravenous immunoglobulin (IVIg) is occasionally used.

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

Localized treatment of ulcers and blisters may include soothing or drying lotions, wet dressings, or similar measures.

Expectations (prognosis):

If not treated, pemphigus vulgaris is usually fatal. Generalized infection is the most frequent cause of death. Treated, the disorder tends to be chronic in most cases. Side effects of treatment may be severe or disabling.

Complications:

• Side effects of systemic medications
• Secondary bacterial, viral, or fungal infection of the skin
• Spread of infection through the bloodstream ( sepsis )
• Loss of extensive amounts of body fluids
• Loss of electrolytes, electrolyte disturbances

Calling your health care provider:

Any unexplained blisters should always be examined by your physician.

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

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

• Review Date: 7/27/2006
• Reviewed By: Shlomit Halachmi, MD, PhD, Private Practice Specializing in Dermatology, Somerville, MA. Review provided by VeriMed Healthcare Network.

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