Risk Factors for Mucosal Involvement in Bullous Pemphigoid and the Possible Mechanism: A Review.

Xinyi Chen,Hongzhong Jin,Li Li,Wenlin Zhao

doi:10.3389/fmed.2021.680871

Abstract

Bullous pemphigoid (BP) is the most common type of autoimmune bullous disease and is characterized by the presence of circulating anti-BP180 and/or anti-BP230 autoantibodies. Patients with BP often present with tense blisters and erythema, mainly on the trunk and limbs, but a few patients also have mucosal involvement. In this article, we discuss the fact that BP patients with mucosal involvement tend to have more serious conditions and their disease is more difficult to control. Potential risk factors for mucous involvement include earlier age at onset, drugs such as dipeptidyl peptidase-4 inhibitors, cancer, and blood/serum biomarkers, including lower eosinophil count, higher erythrocyte sedimentation rate, IgG autoantibodies against both the NH2- and COOH-termini of BP180, and the absence of anti-BP230 antibodies. IgA and C3 deposition at the dermo-epidermal junction may also be present. Understanding these risk factors may benefit earlier diagnosis of these patients and promote the development of novel treatments. What's more, it's helpful in deeper understanding of BP development and the relationship between BP and mucous membrane pemphigoid (MMP).

Highlights

Bullous pemphigoid (BP) is the most common type of autoimmune bullous disease and is characterized by the presence of circulating anti-BP180 and/or anti-BP230 autoantibodies
Kirtschig et al found that most BP patients with oral involvement had higher titers of BP180 antibodies than patients without oral lesions, as detected using the indirect immunofluorescent technique, indicating that skins and mucosa may express different epitopes and these two kinds of patients produce autoantibodies to different epitopes [28]
BP patients with mucosal lesions tend to be younger, have more severe disease, and are less sensitive to conventional treatment than patients without mucosal involvement, which underscores the need for early diagnosis and effective therapy

Summary

Introduction

Bullous pemphigoid (BP) is the most common type of autoimmune bullous disease and is characterized by the presence of circulating anti-BP180 and/or anti-BP230 autoantibodies. The diagnosis of MMP with generalized blisters and of BP with extensive mucosal involvement remains challenging especially in patients with circulating anti-BP180 autoantibodies. Patients with mucosal involvement have a higher incidence of treatment with DPP-4 inhibitors [12].

Results

Conclusion