Desmogleins 1, 3, and E-cadherin immunohistochemical expression within mucocutaneous pemphigus vulgaris

Abstract

Introduction: pemphigus vulgaris (PV) is an autoimmune condition characterized by the loss of adhesion between the epithelial cells and blister formation. The production of autoantibodies against desmosomal proteins, namely, desmoglein (DSG) 1 and DSG3, is considered a main event of PV. A full understanding of the role of adhesion molecules in the pathogenesis of PV is not fully elucidated yet. This study aimed to evaluate and correlate the immunohistochemical expression of E-cadherin (E-cad), DSG1, and DSG3 proteins in oral and skin PV.

Methods: this study was a retrospective analysis study. Positive PV cases were stained with anti-E-cad, anti-DSG1, and anti-DSG3 antibodies. The expression of each marker was determined by two pathologists according to an established scoring system: (E-cad: negative, weak, moderate, and strong), (DSG1: negative, weak, and strong), and (DSG3: negative and positive). The Chi-square and Pearson´s correlation tests were used to statistically analyze the data.

Results: forty-three biopsies (26 skin and 17 oral tissue samples) from 22 males and 21 female PV patients were included. The median age was 40.50 years. In total, the immunohistochemical expression was negative for DSG3, E-cad, and DSG1 in 81.4%, 18.5%, and 16.4%, respectively. DSG1 expression was significantly higher in males than females. A statistically significant correlation was found between E-cad and DSG3 expressions.

Conclusion: a significant difference in the expression of markers of both oral and skin PV was absent. Downregulation of DSG3 expression was the hallmark feature that also showed a positive correlation with E-cad expression.

Keywords: E-cadherin; Pemphigus vulgaris; desmoglein1; desmoglein3.

Figure 1

the expression of desmoglein 1 (DSG1), desmoglein 3 (DSG3), and E-cadherin (E-cad) in multiple pemphigus vulgaris cases: A) positive DSG3 expression; B) negative DSG3 expression; C) strong positive DSG1 expression; D) weak positive DSG1 expression; E) negative DSG1 expression; F) strong E-cad expression; G) moderate E-cad expression; H) mild E-cad expression; and I) negative E-cad expression

Figure 2

correlation of cellular adhesion molecules, E-cadherin (E-cad) is positively and significantly associated with desmoglein (DSG) 3 but not with DSG1; in addition, DSG1 and DSG3 did not show any significant correlation (*p-value = 0.003 by Spearman’s correlation test)