Pemphigus vulgaris

Definition

Pemphigus vulgaris is an autoimmune disorder that involves blistering and sores (erosions) of the skin and mucus membranes.

Causes

Pemphigus is an autoimmune disorder. The immune system produces antibodies against specific proteins in the skin and mucus membranes. These antibodies break the bonds between skin cells. This leads to the formation of a blister. The exact cause is unknown.

Sometimes pemphigus is caused by certain medications, although this is rare. Medications that may cause this condition include:

• A drug called penicillamine, which removes certain materials from the blood (chelating agent)
• Blood pressure medications called ACE inhibitors

Pemphigus is uncommon. It almost always occurs in middle-aged or older people.

Symptoms

About 50% of people with this condition first develop painful blisters and sores in the mouth, followed by skin blisters. Skin sores may come and go.

The skin sores may be described as:

• Draining
• Oozing
• Crusting
• Peeling or easily detached

They may be located:

• In the mouth
• On the scalp, trunk, or other skin areas

Exams and Tests

The skin separates easily when the surface of unaffected skin is rubbed sideways with a cotton swab or finger. This is called a positive Nikolsky's sign.

A skin biopsy is usually done to confirm the diagnosis.

Treatment

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

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.

Outlook (Prognosis)

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.

Possible Complications

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

When to Contact a Medical Professional

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

References

Habif TP. Vesicular and bullous diseases. In: Habif TP, ed. Clinical Dermatology. 5th ed. Philadelphia, Pa: Mosby Elsevier;2009:chap 16.

Baroni A, Lanza A, Cirillo N, Brunetti G, Ruocco E, Ruocco V. Vesicular and bullous disorders: pemphigus. Dermatol Clin. 2007;25(4):597-603.

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