Aoi Fukuda

Four cases of mucous membrane pemphigoid with clinical features of oral lichen planus

Mucous membrane pemphigoid (MMP) and oral lichen planus (OLP) show similar clinical features on the oral mucosa. As clinical manifestations of oral mucosal lesions, MMP shows blisters and erosions, whereas OLP shows lace‐like whitish lesions in an annular arrangement with erythema and erosions. Histopathologically, MMP shows subepithelial bullae with infiltrates of lymphocytes and neutrophils, whereas OLP shows band‐like interface infiltration of lymphocytes with damage in basal cells. However, these two diseases are frequently difficult to distinguish both clinically and histopathologically.

Ten cases of severe oral lichen planus showing granular C3 deposition in oral mucosal basement membrane zone.

BackgroundOral lichen planus (OLP) may show depositions of immunoglobulins and complement components in oral mucosal basement membrane zone (BMZ) in direct immunofluorescence, although these finding are not frequently seen.ObjectiveWe collected and examined ten cases of severe OLP showing granular C3 deposition in BMZ.Materials and MethodsIn addition to clinical, histopathological and direct immunofluorescence assessments, we performed various immuneserological tests, including indirect immunofluorescence of normal human skin and 1M NaCl-split skin, immunoblotting of normal human epidermal and dermal extracts, recombinant proteins of BP180 NC16a and C-terminal domains, concentrated culture supernatant of HaCaT cells and purified human laminin-332, and enzyme-linked immunosorbent assays for BP230 and BP180.ResultsDirect immunofluorescence showed C3 deposition in BMZ exclusively of granular pattern in 7 cases and of both granular and linear patterns in 3 cases. The 10 cases showed no positive reactivity for either IgG or IgA antibodies in any immunoserological tests. Detailed analyses of clinical, histopathological and immunological findings revealed striking female prevalence, although other parameters were in general characteristic of OLP.ConclusionsGranular C3 deposition in oral BMZ may be one of the characteristic features of severe OLP, although mechanisms for C3 deposition and its pathogenic role in OLP are currently unknown.

A case of mucous membrane pemphigoid with IgG antibodies against the β3 and γ2 subunits of laminin‐332, and the C‐terminal domain of BP180

Mucous membrane pemphigoid (MMP) is a rare autoimmune subepidermal blistering disease that predominantly affects the mucous membranes, including the ocular, oral, gingival, nasopharyngeal, esophageal, and genital mucosae. MMP usually results in permanent scarring of the affected area, which can eventually lead to serious complications including blindness and upper aerodigestive tract stenosis. Target autoantigens identified in MMP include two major autoantigens, the C-terminal domain of BP180 (BPAG2 or type XVII collagen) and laminin-332, and the 120-kDa ectodomain of BP180, BP230, laminin-311, type VII collagen, a6 integrin, b4 integrin and uncein, and three as unidentified epithelial proteins of 45, 168, and 120 kDa. We herein report a case of MMP with IgG antibodies against the b3 and c2 subunits of laminin-332, as well as the C-terminal domain of BP180. Case report