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What is pemphigus vulgaris?

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

How is pemphigus vulgaris diagnosed?

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.

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

What to expect?

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

How is pemphigus vulgaris treated?

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:
  • IVIG
  • 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.

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