Pemphigus vulgaris: a case-based update

Pemphigus vulgaris (PV) is an autoimmune disease accounting for 80% of all cases of pemphigus. Before the advent of corticosteroid therapy, pemphigus was fatal, with a mortality rate of up to 75% in the first year. It is still a serious disorder, but the 5% to 10% mortality rate is now primarily due to the side effects of therapy. In 75% to 80% of cases, PV lesions first appear in the oral cavity. Dentists are therefore in a unique position to recognize the oral manifestations of the disease, allowing early diagnosis and initiation of treatment. The diagnosis is based on pathological examination and immunofluorescence testing. Systemic corticosteriods and steroid-sparing agents are the mainstays of treatment; topical corticosteroids may also be used to accelerate healing of persistent oral lesions. This article describes a 71-year-old woman with multiple chronic ulcers in the oral cavity, in whom PV was diagnosed 4 months after the symptoms first appeared. The article also reviews the current literature on diagnosis and treatment of the condition.