Abstract
Mucous membrane pemphigoid (MMP) is a mucous membrane-dominated autoimmune subepithelial blistering disease that is caused by autoantibodies against various autoantigens in basement membrane zone (BMZ) proteins, including collagen XVII (COL17). Clinicians face diagnostic problems in detecting circulating antibodies and targeted antigens in MMP. The diagnostic difficulties are mainly attributed to the low titers of MMP autoantibodies in sera and to heterogeneous autoantigens. Additionally, no unanimous diagnostic criteria have been drawn for MMP, which can result in delayed diagnoses or misdiagnoses. This review aims to integrate and present currently available data to clarify diagnostic strategies and to present diagnostic criteria for MMP. The ultimate blistering mechanism in MMP has not been elucidated, and such mechanism is especially obscure in COL17-type MMP. In bullous pemphigoid (BP), which is the most common autoimmune subepidermal blistering disease, some patients show oral lesion as well as predominant skin lesions. However, there is no fundamental explanation for the onset of oral lesions in BP. This article summarizes innovative research perspectives on the pathogenesis of oral lesions in pemphigoid. Finally, we propose a potential pathogenesis for COL17-type MMP.
Highlights
Mucous membrane pemphigoid (MMP) refers to mucous membrane-dominated autoimmune subepithelial blistering diseases [1,2,3,4]
MMP is caused by autoantibodies against various autoantigens in the basement membrane zone (BMZ), including collagen XVII (COL17, called BP180) [5], BP230 [6], laminin 332 [7,8,9], integrin α6/β4 [10,11,12], and collagen VII (COL7) [13, 14]
To identify circulating autoantibodies to the BMZ, indirect immunofluorescence (IIF) with normal human skin as the substrate is usually performed, but autoantibodies are detected in only 17–53% of MMP cases [5, 17, 18, 29]
Summary
Specialty section: This article was submitted to Autoimmune and Autoinflammatory. Citation: Kamaguchi M and Iwata H (2019) The Diagnosis and Blistering Mechanisms of Mucous Membrane Pemphigoid. Mucous membrane pemphigoid (MMP) is a mucous membrane-dominated autoimmune subepithelial blistering disease that is caused by autoantibodies against various autoantigens in basement membrane zone (BMZ) proteins, including collagen XVII (COL17). The diagnostic difficulties are mainly attributed to the low titers of MMP autoantibodies in sera and to heterogeneous autoantigens. No unanimous diagnostic criteria have been drawn for MMP, which can result in delayed diagnoses or misdiagnoses. The ultimate blistering mechanism in MMP has not been elucidated, and such mechanism is especially obscure in COL17-type MMP. In bullous pemphigoid (BP), which is the most common autoimmune subepidermal blistering disease, some patients show oral lesion as well as predominant skin lesions. This article summarizes innovative research perspectives on the pathogenesis of oral lesions in pemphigoid. We propose a potential pathogenesis for COL17-type MMP
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