Health Encyclopedia

Pemphigus vulgaris

Definition

Pemphigus vulgaris (PV) is an autoimmune disorder of the skin. It involves blistering and sores (erosions) of the skin and mucous membranes.

Causes

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

In rare cases, pemphigus is caused by some medicines, including:

A medicine called penicillamine, which removes certain materials from the blood (chelating agent)
Blood pressure medicines called ACE inhibitors
Nonsteroidal anti-inflammatory drugs (NSAIDs)

Pemphigus is uncommon. It most often occurs in middle-aged or older people.

Symptoms

About 50% of people with this condition first develop painful blisters and sores in the mouth. This is 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 and down the throat
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 sign.

A skin biopsy and blood tests are often done to confirm the diagnosis.

Treatment

Severe cases of pemphigus may need wound management, similar to the treatment for severe burns. People with PV 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 medicines to control or prevent infections
Fluids and electrolytes given through a vein (IV) if there are severe mouth ulcers
IV feedings if there are severe mouth ulcers
Numbing (anesthetic) mouth lozenges to reduce mouth ulcer pain
Pain medicines 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 medicine called dapsone
Corticosteroids
Medicines containing gold
Medicines that suppress the immune system (such as azathioprine, methotrexate, cyclosporine, cyclophosphamide, mycophenolate mofetil, or rituximab)

Antibiotics may be used to treat or prevent infection. Intravenous immunoglobulin (IVIg) is occasionally used.

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

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

Outlook (Prognosis)

Without treatment, this condition can be 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

Complications of PV include:

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

When to Contact a Medical Professional

Your health care provider should examine any unexplained blisters.

Contact your provider if you have been treated for PV and you develop any of the following symptoms:

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

References

Amagai M. Pemphigus. In: Bolognia JL, Schaffer JV, Cerroni L, eds. Dermatology. 4th ed. Philadelphia, PA: Elsevier; 2018:chap 29.

Dinulos JGH. Vesicular and bullous diseases. In: Dinulos JGH, ed. Habif's Clinical Dermatology. 7th ed. Philadelphia, PA: Elsevier; 2021:chap 16.

James WD, Elston DM, Treat JR, Rosenbach MA, Neuhaus IM. Chronic blistering dermatoses. In: James WD, Elston DM, Treat JR, Rosenbach MA, Neuhaus IM, eds. Andrew's Diseases of the Skin. 13th ed. Philadelphia, PA: Elsevier; 2020:chap 21.

Patterson JW. The vesiculobullous reaction pattern. In: Patterson JW, ed. Weedon's Skin Pathology. 5th ed. Philadelphia, PA: Elsevier; 2021:chap 7.

Review Date:5/31/2022
Reviewed By:Elika Hoss, MD, Assistant Professor of Dermatology, Mayo Clinic, Scottsdale, AZ. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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