Acantholysis may precede elevation of circulating anti‐desmoglein 3 antibody levels in pemphigus vulgaris presenting with desquamative gingivitis

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.

Retrospective study of gingival involvement in pemphigus: A difficult to treat phenomenon

“…Being negative for anti-Dsg3 was also reported in some PV patients with DG; however, of five patients with undetectable anti-Dsg3 antibodies at the diagnosis, during the follow-up, anti-Dsg3 titters increased and subsequently became positive in three cases. 22 Considering the fact that the acantholysis occurred in the patients with negative anti-Dsgs, similar to half of our cases, it has been speculated that anti-Dsg3 antibodies detected by ELISA could be the witnesses of disease rather than the reason for PV acantholysis and DG in PV patients. Another hypothesis is that these lesions initially might be developed due to autoantibodies binding to the desmosome, while the lesions remained activated, even in the absence of anti-Dsg1/3 autoantibodies.…”

“…This suggests other pathogenic signaling pathways than Dsg-3-specific autoantibodies. 22 Thus, one hypothesis for this is the involvement of non-Dsg autoantibodies, such as anti-Desmocollins. Being negative for anti-Dsg3 was also reported in some PV patients with DG; however, of five patients with undetectable anti-Dsg3 antibodies at the diagnosis, during the follow-up, anti-Dsg3 titters increased and subsequently became positive in three cases.…”

“…Indeed, it has been reported that the anti‐Dsg3 antibody measured by ELISA was negative in some PV patients presenting with DG. This suggests other pathogenic signaling pathways than Dsg‐3‐specific autoantibodies 22 . Thus, one hypothesis for this is the involvement of non‐Dsg autoantibodies, such as anti‐Desmocollins.…”

Revisiting the question of pemphigus classification

“…Мы считаем это нецелесообразным по следующим причинам: во-первых, исследования 2019 г. показали, что при изолированном поражении слизистой оболочки полости рта при вульгарной пузырчатке могут выявляться аутоантитела к Dsg-3 [17], во-вторых, в подавляющем большинстве случаев развиваются поражения кожи на фоне вовлечения слизистых оболочек, в-третьих, лечение этих форм практически не отличается.…”

Diagnostic value of cytology in oral ulcer and comparison with histopathology and direct immunofluorescence

“…Pemphigus vulgaris (PV) is one of the autoimmune diseases that manifests by skin and mucosal bullae 1,2 . Its usual first manifestation is development of nonhealing and painful erosions of oral mucosa 3,4 . Histopathology and direct immunofluorescence (DIF) are reliable and definitive tests for the diagnosis of PV 5 .…”