Pemphigus Vulgaris

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

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


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:
• 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.


Tests & Diagnosis

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.