Disease progression from mucosal to mucocutaneous involvement in a patient with desquamative gingivitis associated with pemphigus vulgaris

Background: Pemphigus vulgaris (PV) frequently begins with oral lesions and progresses to skin lesions. A patient is described who developed skin lesions during follow-up and whose only initial symptom was desquamative gingivitis (DG). Methods: A 31-year-old woman presented with a 2-month history of painful gingiva. The diagnosis of PV was made according to clinical, histopathological, and immunofluorescent criteria. Topical corticosteroid (0.1% triamcinolone acetonide) was provided for the treatment of DG. Evaluation of the circulating autoantibody titers to desmoglein (Dsg)1 and Dsg3 was conducted by enzyme-linked immunosorbent assay (ELISA). Results: The gingival PV lesions went into remission with the use of topical corticosteroid, although the patient experienced occasional recurrent oral lesions that required retreatment. She had regular follow-ups and remained relatively stable for several months. However, relapse and worsening of the oral lesions and the onset of skin lesions occurred after 26 months. Using ELISA, a change in the autoantibody profile corresponding to the transition from mucosal PV to mucocutaneous PV was confirmed. In all ELISA studies conducted throughout the course of the patient's disease, the Dsg3 ELISA was consistently high ranging from 150 to 200. However, the Dsg1 ELISA remained low, ranging from 10 to 30. After 26 months, Dsg3 (index value of 150) and Dsg1 (index value of 114) ELISA levels were elevated, consistent with the transition to mucocutaneous PV. Conclusions: In cases in which the lesions are limited to the oral cavity, PV sometimes may be managed successfully using only topical corticosteroids. However, it may not be possible to reduce the circulating Dsg autoantibody titers without systemic immunosuppression. The sustained high Dsg3 antibody level may cause "epitope spreading" and induce skin lesions. It may be prudent to determine post-treatment levels of Dsg using ELISA and, in consultation with the physician, recommend the addition of systemic therapy if Dsg3 levels remain elevated.