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[自身免疫性大疱性疾病中的黏膜异常]

[Mucosal anomalies in autoimmune bullous diseases].

作者信息

Meijer J M, Vissink A

出版信息

Ned Tijdschr Tandheelkd. 2023 May;130(5):237-241. doi: 10.5177/ntvt.2023.05.22090.

Abstract

Mucosal anomalies are frequently seen in autoimmune bullous diseases, particularly in pemphigus vulgaris and mucous membrane pemphigoid. The blistering, erosions, ulceration or erythema may present anywhere on the oral mucosa, but also on other mucosal sites. A differential diagnosis is needed of (erosive) oral lichen planus, systemic autoimmune disease, inflammatory bowel diseases, chronic graft-versus-host disease, infectious causes, Behçet's syndrome and recurrent aphthous stomatitis. A quick diagnosis and initiation of adequate treatment are important because of the potential severity of the disease and to prevent complications due to cicatrization. Besides a biopsy for histopathological analysis, a perilesional biopsy for direct immunofluorescence microscopy and immunoserological tests are needed for diagnosis of pemphigus or pemphigoid. In addition to a mucosal biopsy, a biopsy for direct immunofluorescence of the skin can contribute to a diagnosis of a bullous disease. Besides topical corticosteroids, immunosuppressive treatment is often required for treating autoimmune bullous diseases, such as treatment with rituximab in patients with pemphigus.

摘要

黏膜异常在自身免疫性大疱性疾病中很常见,尤其是寻常型天疱疮和黏膜类天疱疮。水疱、糜烂、溃疡或红斑可能出现在口腔黏膜的任何部位,也可能出现在其他黏膜部位。需要对(糜烂性)口腔扁平苔藓、系统性自身免疫性疾病、炎症性肠病、慢性移植物抗宿主病、感染性病因、白塞病和复发性阿弗他口炎进行鉴别诊断。由于疾病的潜在严重性以及为防止瘢痕形成引起的并发症,快速诊断并开始适当治疗很重要。除了进行组织病理学分析的活检外,还需要进行病损周围活检以进行直接免疫荧光显微镜检查和免疫血清学检测,用于诊断天疱疮或类天疱疮。除了黏膜活检外,皮肤直接免疫荧光活检有助于诊断大疱性疾病。除局部使用皮质类固醇外,治疗自身免疫性大疱性疾病通常还需要免疫抑制治疗,例如在天疱疮患者中使用利妥昔单抗进行治疗。

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