自身免疫性大疱性疾病的粘膜异常。

J M Meijer, A Vissink
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引用次数: 0

摘要

黏膜异常常见于自身免疫性大疱性疾病,特别是寻常型天疱疮和粘膜类天疱疮。起泡、糜烂、溃疡或红斑可出现在口腔粘膜的任何部位,也可出现在其他粘膜部位。需要鉴别诊断(糜烂性)口腔扁平苔藓、全身自身免疫性疾病、炎症性肠病、慢性移植物抗宿主病、感染性原因、behet综合征和复发性口疮性口炎。由于该疾病的潜在严重性和防止因愈合引起的并发症,快速诊断和开始适当治疗非常重要。诊断天疱疮或类天疱疮,除了进行组织病理学分析的活检外,还需要进行直接免疫荧光显微镜和免疫血清学检查的病灶周围活检。除了粘膜活检外,皮肤直接免疫荧光活检也有助于大疱性疾病的诊断。除了局部皮质类固醇外,免疫抑制治疗通常需要用于治疗自身免疫性大疱性疾病,例如天疱疮患者的利妥昔单抗治疗。
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[Mucosal anomalies in autoimmune bullous diseases].

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.

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