Pemphigus vulgaris is an autoimmune disorder that involves blistering of the skin and mucous membrane.
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.
About 50% of cases begin with blisters in the mouth, followed by skin blisters. Skin lesions may come and go.
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.
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.