Bhandari Adhyatm, De Dipankar, Shah Shikha, Chatterjee Debajyoti, Kumar Vinod, Mahajan Rahul, Handa Sanjeev
Department of Dermatology, Venereology, and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Indian Dermatol Online J. 2024 Nov 5;16(3):370-380. doi: 10.4103/idoj.idoj_253_24. eCollection 2025 May-Jun.
Autoimmune bullous diseases (AIBDs) are a group of illnesses characterized by autoantibodies targeting adhesion molecules in the skin and mucosa. Accurate diagnosis of the specific subtype of AIBD is crucial for effective management and predicting prognosis, especially in cases with an increased risk of malignancy. However, differentiating between subtypes can be challenging due to overlapping symptoms.
Direct immunofluorescence microscopy (DIF) detects bound antibodies in perilesional tissue biopsies and provides details about the probable site of autoantibody deposition within the skin/mucosae, immunoglobulin type, and pattern of antibody deposition. Indirect immunofluorescence (IIF) microscopy with organ substrate is a minimally invasive serological test that detects circulating autoantibodies. Enzyme-linked immunosorbent assay (ELISA) quantifies serum autoantibodies against specific autoantigens. Quantitative ELISA is useful for diagnosis, monitoring therapy, and assessing disease activity. Commercially available ELISA kits, including the multi-variant ones, can detect antibodies associated with AIBDs. BIOCHIP is a technique based on IIF that offers a sensitive and specific diagnostic alternative to ELISA. It uses microarrays with multiple antigenic substrates to simultaneously screen common AIBDs. The BIOCHIP slides contain different substrates, allowing the identification of multiple types of autoantibodies in a single test.
While these diagnostic tests offer valuable insights into target antigens, antibody patterns, and disease subtypes, it is important to note that the availability of these tests is limited in most centers across India. This limitation can be attributed to factors such as the relatively higher cost of these investigations, challenges related to the stability of immuno-reactants, and a shortage of trained personnel capable of performing such tests.
This review discusses the diagnosis of AIBDs based on resources available in India, as of today. It also provides with practically applicable diagnostic algorithms for pragmatic diagnosis of AIBDs in Indian scenario.
自身免疫性大疱性疾病(AIBD)是一组以针对皮肤和黏膜黏附分子的自身抗体为特征的疾病。准确诊断AIBD的特定亚型对于有效管理和预测预后至关重要,尤其是在恶性肿瘤风险增加的病例中。然而,由于症状重叠,区分亚型可能具有挑战性。
直接免疫荧光显微镜检查(DIF)可检测皮损周围组织活检中的结合抗体,并提供有关自身抗体在皮肤/黏膜内可能沉积部位、免疫球蛋白类型和抗体沉积模式的详细信息。使用器官底物的间接免疫荧光(IIF)显微镜检查是一种微创血清学检测方法,可检测循环自身抗体。酶联免疫吸附测定(ELISA)可定量针对特定自身抗原的血清自身抗体。定量ELISA对诊断、监测治疗和评估疾病活动很有用。市售的ELISA试剂盒,包括多变量试剂盒,可检测与AIBD相关的抗体。生物芯片是一种基于IIF的技术,为ELISA提供了一种敏感且特异的诊断替代方法。它使用带有多种抗原底物的微阵列同时筛查常见的AIBD。生物芯片载玻片包含不同的底物,可在一次测试中鉴定多种类型的自身抗体。
虽然这些诊断测试能提供有关靶抗原、抗体模式和疾病亚型的有价值信息,但需要注意的是,在印度的大多数中心,这些测试的可用性有限。这种限制可归因于这些检查成本相对较高、免疫反应物稳定性相关的挑战以及缺乏能够进行此类测试的训练有素的人员等因素。
本综述讨论了截至目前基于印度现有资源的AIBD诊断。它还提供了在印度实际应用的实用诊断算法,用于AIBD的实际诊断。