Sood Ridhi, Chatterjee Debajyoti, De Dipankar, Nahar Saikia Uma, Mahajan Rahul, Handa Sanjeev, Dass Radotra Bishan
Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Department of Dermatology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Indian J Dermatol Venereol Leprol. 2023 Aug 10:1-6. doi: 10.25259/IJDVL_20_2023.
Background Direct immunofluorescence (DIF) is essential for the diagnosis of sub-epidermal immunobullous diseases (SIBD). Bullous pemphigoid (BP), a sub-epidermal immunobullous disease, shows linear IgG and C3 deposition along the dermo-epidermal junction by DIF. However, similar histological and DIF findings are also seen in epidermolysis bullosa acquisita (EBA). High-power examination of antibody deposition by DIF in a "u" or "n" serrated pattern can help differentiate these two entities. Aims/Objectives The aim of this study was to determine the diagnostic accuracy of serration patterns in IgG-mediated sub-epidermal immunobullous disease. Methods All cases of IgG-mediated sub-epidermal immunobullous disease diagnosed over the past 2 years and 9 months period and confirmed serologically, were included. Examination of the serration pattern in DIF was assessed on oil emersion. Salt split skin indirect immunofluorescence (SSS IIF), BP180 enzyme-linked immunosorbent assay (ELISA), profile ELISA and BIOCHIP mosaic were performed, wherever available. Results This study included 74 cases of bullous pemphigoid, eight cases of mucus membrane pemphigoid (MMP) and one case of epidermolysis bullosa acquisita. The characteristic zigzag "n" pattern was visualised in 66 out of 82 cases (80.5%) of the pemphigoid group (BP + MMP); the single epidermolysis bullosa acquisita case showed the "u" serrated pattern. No statistical correlation was seen between serration pattern and BP180 positivity by ELISA (P = 0.05). Limitations The study is limited by the single case of epidermolysis bullosa acquisita (which could be due to rarity of this disease in north Indian population due to genetic variation), lack of detailed serological investigations and immunoblot in all cases. Conclusion Serration pattern analysis is an easy-to-interpret and highly useful technique for characterisation of sub-epidermal immunobullous diseases.
背景 直接免疫荧光法（DIF）对于表皮下免疫性大疱病（SIBD）的诊断至关重要。大疱性类天疱疮（BP）是一种表皮下免疫性大疱病，通过DIF显示沿真皮-表皮交界处有线性IgG和C3沉积。然而，在获得性大疱性表皮松解症（EBA）中也可见类似的组织学和DIF表现。通过DIF对抗体沉积进行高倍检查呈“u”形或“n”形锯齿状模式有助于区分这两种疾病。目的 本研究的目的是确定IgG介导的表皮下免疫性大疱病中锯齿状模式的诊断准确性。方法 纳入过去2年9个月期间诊断为IgG介导的表皮下免疫性大疱病且血清学确诊的所有病例。在油浸下评估DIF中的锯齿状模式。如有条件，进行盐裂皮肤间接免疫荧光法（SSS IIF）检测、BP180酶联免疫吸附测定（ELISA）、轮廓ELISA和生物芯片镶嵌分析。结果 本研究包括74例大疱性类天疱疮、8例黏膜类天疱疮（MMP）和1例获得性大疱性表皮松解症。在类天疱疮组（BP + MMP）的82例病例中有66例（80.5%）可见特征性的锯齿状“n”形模式；1例获得性大疱性表皮松解症病例显示“u”形锯齿状模式。ELISA检测的锯齿状模式与BP180阳性之间未见统计学相关性（P = 0.05）。局限性 本研究受限于仅1例获得性大疱性表皮松解症病例（这可能是由于印度北部人群中该疾病因基因变异而罕见），且所有病例缺乏详细的血清学检查和免疫印迹分析。结论 锯齿状模式分析是一种易于解释且对表皮下免疫性大疱病特征化非常有用的技术。
