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Pemphigus vulgaris

 

Definition

Pemphigus vulgaris is an autoimmune disorder that involves blistering of the skin and mucous membrane.

Causes

Pemphigus is an autoimmune disorder. The immune system produces antibodies against specific proteins in the skin and mucous membrane. These antibodies create a reaction that cause skin cells to separate. The exact cause is unknown.

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

  • Blood pressure medications called ACE inhibitors
  • Chelating agents such as penicillamine, which remove certain materials from the blood

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

Symptoms

About 50% of cases begin with blisters in the mouth, followed by skin blisters. Skin lesions may come and go.

The skin lesions 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
Signs 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 lesion biopsy can help confirm the diagnosis.

Support Groups

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.

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:

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

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.

Prevention

References

Baroni A, Lanza A, Cirillo N, Brunetti G, Ruocco E, Ruocco V. Vesicular and Bullous Disorders: Pemphigus. Dermatol Clin. 2007 Oct;25(4):597-603, ix.


Review Date: 7/11/2008
Reviewed By: Michael Lehrer, M.D., Department of Dermatology, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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