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.

Treatment may involve:

• Fluids, proteins, and electrolytes given through a vein (IV)
• IV feedings if there are severe mouth ulcers
• Anesthetic (numbing) mouth lozenges to reduce mouth ulcer pain
• Antibiotics and antifungal medications to control or prevent infections

Body-wide (systemic) therapy is needed to control pemphigus and should be started as early as possible. Systemic treatment includes corticosteroids, medications containing gold, an anti-inflammatory drug called dapsone, and medications that suppress the immune system (such as azathioprine, methotrexate, cyclosporin, cyclophosphamide, or mycophenolate mofetil). 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 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):

Without treatment, this condition is usually deadly. Generalized infection is the most frequent cause of death.

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

Complications:

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

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