Muramatsu Ken, Nishie Wataru, Natsuga Ken, Fujita Yasuyuki, Iwata Hiroaki, Yamada Tamaki, Yamashita Emi, Asaka Takuya, Shimizu Hiroshi
Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
Department of Oral and Maxillofacial Surgery, Division of Oral Pathobiological Science, Sapporo, Japan.
J Dermatol. 2016 Nov;43(11):1350-1353. doi: 10.1111/1346-8138.13493. Epub 2016 Jul 26.
Oral lichen planus (OLP) is a chronic inflammatory disorder of the oral mucosa of unknown etiology. Clinically, the erosive type of OLP (erosive OLP) can show features similar to those of pemphigus vulgaris (PV), an autoimmune blistering disorder in which desmoglein (Dsg)3 is targeted. In addition to clinical and histopathological findings, immunological studies, including direct immunofluorescence (IF), indirect IF and enzyme-linked immunosorbent assay (ELISA) that detect autoantibodies to Dsg3, are helpful in differentiating erosive OLP from PV. Here, we show two cases of erosive OLP with autoantibodies to Dsg3. Patient 1 was a 68-year-old woman with chronic erosions of the oral mucosa, in which elevated levels of immunoglobulin (Ig)G autoantibodies to Dsg1 and Dsg3 were detected by ELISA. Patient 2 was an 85-year-old woman with white striae with erosions on the lateral sides of the buccal mucosa with elevated levels of IgG autoantibodies to Dsg3 detected by ELISA. Histopathological findings from both cases showed lichenoid dermatitis, and both direct and indirect IF showed no tissue-bound IgG autoantibodies. From these findings, the diagnosis of erosive OLP was made. Immunological assays revealed both cases to have IgG-directing calcium-independent linear epitopes on Dsg3, which are suggestive of non-pathogenic autoantibodies. In addition, autoantibodies to Dsg3 in patient 2 reacted with a prosequence-possessing precursor form of Dsg3 but not with the mature form of the molecule. The present study suggests that erosive OLP may develop anti-Dsg3 autoantibodies, which should be carefully assessed.
口腔扁平苔藓(OLP)是一种病因不明的口腔黏膜慢性炎症性疾病。临床上,糜烂型OLP(糜烂性OLP)可表现出与寻常型天疱疮(PV)相似的特征,PV是一种自身免疫性水疱性疾病,其靶抗原为桥粒芯糖蛋白(Dsg)3。除临床和组织病理学检查结果外,包括直接免疫荧光(IF)、间接IF和酶联免疫吸附测定(ELISA)等检测抗Dsg3自身抗体的免疫学研究,有助于糜烂性OLP与PV的鉴别诊断。在此,我们报告2例伴有抗Dsg3自身抗体的糜烂性OLP病例。病例1为一名68岁女性,患有口腔黏膜慢性糜烂,通过ELISA检测发现其抗Dsg1和Dsg3的免疫球蛋白(Ig)G自身抗体水平升高。病例2为一名85岁女性,颊黏膜外侧有白色条纹伴糜烂,通过ELISA检测发现其抗Dsg3的IgG自身抗体水平升高。两例病例的组织病理学检查结果均显示为苔藓样皮炎,直接和间接IF均未显示组织结合的IgG自身抗体。根据这些结果,诊断为糜烂性OLP。免疫学检测显示,两例病例的Dsg3上均有IgG导向的钙非依赖性线性表位,提示为非致病性自身抗体。此外,病例2中抗Dsg3自身抗体与具有前导序列的Dsg3前体形式反应,但不与该分子的成熟形式反应。本研究提示,糜烂性OLP可能产生抗Dsg3自身抗体,对此应进行仔细评估。