Oral and cutaneous pemphigus vulgaris: an atypical clinical presentation

Pemphigus vulgaris (PV) is a rare painful and blistering autoimmune mucocutaneous disorder that appears in middle-aged adults. Oral lesions typically precede cutaneous involvement and tend to be more recalcitrant to therapy. The objective of this article is to present a case of oral and cutaneous PV in an atypical patient, a 23-year-old woman. The case was distinguished by the patient's age, which was 2 to 3 decades younger than the reported mean age of onset, and its coincidence with celiac disease, an immunopathologic process rarely seen in association with PV. Intravenous administration of the monoclonal antibody rituximab provided rapid clinical improvement in the cutaneous lesions and gradual improvement in the oral lesions after 2 infusions. Dental practitioners should remain vigilant for oral manifestations of dermatologic disease and refer affected patients to appropriate healthcare providers for long-term management.