Abstract
Mucous membrane pemphigoid (MMP) is a rare organ-specific autoimmune subepithelial blistering disease with predominantly mucosal erosions, most frequently affecting the gingiva. Erosions in the oral cavity usually result in markedly decreased quality of life. The major autoantigens are BP180 and laminin332, which are components of basement membrane proteins in the skin and mucosa. Diagnosis is usually difficult due to histological destruction of the tissue and low autoantibody titers. In this study, we evaluated the diagnostic value of direct immunofluorescence (DIF) using non-lesional buccal mucosa in seven cases of MMP. In all seven patients, gingival lesions were clinically observed, and in one of the seven patients, buccal lesions were also clinically observed. First, we performed DIF to detect tissue-bound autoantibodies and complement. DIF from non-lesional buccal mucosa revealed linear deposits of IgG and C3 at the basement membrane zone in all cases. To detect autoantibodies, indirect immunofluorescence (IIF), BP180-NC16A ELISA and immunoblotting were performed. Surprisingly, circulating autoantibodies were unable to be detected in any of the cases by ELISA, IIF, or immunoblotting. Furthermore, histological separation was observed in one patient. In conclusion, DIF using non-lesional buccal mucosa was found to be superior to histological and serological tests for diagnosing mucous membrane pemphigoid. The procedure is technically easy and has high diagnostic value.
Highlights
The prevalence and incidence of autoimmune disorders are increasing, with many people suffering from such disorders
Autoimmune subepidermal blistering diseases, e.g., bullous pemphigoid, mucous membrane pemphigoid (MMP) and epidermolysis bullosa acquisita, are organ-specific autoimmune disorders that are characterized by autoantibodies to components of the skin basement membrane zone (BMZ) [1,2,3,4]
Patients were selected according to the following criteria: [1] clinically, MMP was suspected and [2] direct immunofluorescence (DIF) was performed on non-lesional buccal mucosa
Summary
The prevalence and incidence of autoimmune disorders are increasing, with many people suffering from such disorders. MMP shows predominant mucosal involvement, most frequently affecting the oral cavity, followed by the conjunctiva, Abbreviations: MMP, mucous membrane pemphigoid; BMZ, basement membrane zone; IIF, indirect immunofluorescence; DIF, direct immunofluorescence. Autoantibodies are detected in more than 80% of cases in bullous pemphigoid, which is an autoimmune subepidermal blistering disease in which BP180 is targeted [10, 11] This difference tends to be due to the low titers of the autoantibodies in MMP [4]. Histological study fails to show junctional separation because of tissue destruction in the fragile oral mucosa For these reasons, it frequently takes time to make diagnose MMP and start treatment. The sections were incubated with sera from dilutions of 1:10 to 1:320 for 45 min at 37°C, followed by incubation with 1:100 diluted FITC-conjugated anti-human IgG.
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