Abstract
Pemphigus vulgaris (PV) is an autoimmune, blistering disease that affects the mucosa and skin. The current theory favors the concept that anti‐desmoglein (Dsg) 3 autoimmunity is the only pathogenic event needed to induce acantholysis. However, a few cases of active PV in the oral cavity had no detectable anti‐Dsg 3 antibody. The aim of this study was to evaluate the differences in clinical and laboratory findings, whether or not the anti‐Dsg 3 antibodies were present. This study was based on a retrospective review of 10 PV cases. The evaluation of the circulating autoantibody titers to Dsg 3 was conducted by using enzyme‐linked immunosorbent assay (ELISA). An index value of 20 or more was used as the cutoff for a positive reaction. Only five of the 10 PV cases had a positive Dsg 3 ELISA. There were no differences in clinical, cytological, histopathological, and direct immunofluorescence findings, whether or not the anti‐Dsg 3 antibodies were present. Of the five patients with a negative reaction at the time of diagnosis, the Dsg 3 ELISA became positive in the follow‐up period in three cases. In the remaining two cases, the Dsg 3 ELISA was consistently negative for 18 months. Dsg 3 ELISA was negative early in some PV cases. Therefore, PV acantholysis may precede the elevation of circulating anti‐Dsg 3 antibody levels. The diagnosis of PV should be considered based on comprehensive clinical, histopathological, and immunofluorescent criteria.
Highlights
Pemphigus vulgaris (PV) is an autoimmune blistering disease that affects mucous membranes and the skin
Gingival Nikolsky's sign showed a positive reaction in more than 90% of the cases that were diagnosed as PV based on oral lesions (Mignogna et al, 2008)
Cytological, histopathological, and direct immunofluorescence (DIF) findings are consistent with PV, only five of the 10 cases had a positive reaction to the Dsg 3 enzyme‐linked immunosorbent assay (ELISA) at the time of diagnosis (Tables 1 and 2)
Summary
Pemphigus vulgaris (PV) is an autoimmune blistering disease that affects mucous membranes and the skin It rarely occurs, PV is a life‐threatening condition if left untreated, so it is important to diagnose and treat it in its early stages. Gingival Nikolsky's sign showed a positive reaction in more than 90% of the cases that were diagnosed as PV based on oral lesions (Mignogna et al, 2008). This phenomenon can be induced by the application of a shearing force on normal‐appearing gingiva, producing epithelial desquamation. A presumptive diagnosis of PV can be established by confirming the presence of Tzanck cells in the cytologic smear obtained by scraping the gingival lesions (Endo, Rees, Kuyama, et al, 2008)
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