Oral Medicine Unit, Department of Neurosciences, Reproductive and Odontostomatological Sciences, Federico II University of Naples, Naples, Italy.
Periodontol 2000. 2019 Jun;80(1):77-88. doi: 10.1111/prd.12263.
Mucocutaneous blistering autoimmune diseases are a group of systemic, rare, chronic disorders characterized by humoral-mediated immunologic mechanisms against epithelial, basement membrane, and subepithelial tissues. Morbidity and mortality can be completely different among these diseases, with outcome being dependent on an early and accurate diagnosis, systemic comorbidities, and the patient's response to treatment. Definitive diagnosis is based on clinical and histopathologic findings. Because clinical presentations among these diseases are often similar, different immunofluorescence tests and ELISAs are used to confirm the specific diagnosis. Oral mucosa may often be the first site of clinical manifestation from which the disease spreads to other mucosal surfaces and skin. Thus, often dentists and oral medicine specialists may be the first to encounter patients with such diseases. In this review we discuss the most frequent autoimmune vesicobullous disorders, namely pemphigus vulgaris, mucous membrane pemphigoid, bullous pemphigoid, and linear IgA disease.
黏膜水疱性自身免疫性疾病是一组全身性、罕见、慢性疾病，其特征是针对上皮、基底膜和上皮下组织的体液介导免疫机制。这些疾病的发病率和死亡率可能完全不同，其结果取决于早期和准确的诊断、系统性合并症以及患者对治疗的反应。明确的诊断基于临床和组织病理学发现。由于这些疾病的临床表现通常相似，因此使用不同的免疫荧光测试和 ELISA 来确认特定的诊断。口腔黏膜通常是疾病最初表现的部位，然后疾病会扩散到其他黏膜表面和皮肤。因此，牙医和口腔医学专家通常是首先遇到此类疾病患者的人。在这篇综述中，我们讨论了最常见的自身免疫性水疱性疾病，即寻常型天疱疮、黏膜类天疱疮、大疱性类天疱疮和线性 IgA 疾病。
