寻常型天疱疮中桥粒芯糖蛋白 1 和 3 的免疫组化与自身抗体水平和临床严重程度的相关性。
Correlation of desmoglein 1 and 3 immunohistochemistry with autoantibody levels and clinical severity in pemphigus.
机构信息
Department of Pathology, Amrita Institute of Medical Sciences, Kochi, Kerala, India.
Department of Dermatology, Amrita Institute of Medical Sciences, Kochi, Kerala, India.
出版信息
J Cutan Pathol. 2023 Dec;50(12):1104-1109. doi: 10.1111/cup.14501. Epub 2023 Aug 16.
BACKGROUND
Pemphigus is a chronic potentially fatal autoimmune bullous disorder. Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are the two common subtypes. PV is the most common and aggressive type characterized by oral mucosal erosions and cutaneous lesions. PF presents with blisters on the scalp, face, and upper trunk, and spares the mucosae. Direct immunofluorescence (DIF) is the gold standard for diagnosis. Immunohistochemistry (IHC) is an emerging alternate diagnostic tool. In this study, our objectives were to identify the staining patterns of desmoglein 1 (dsg 1) and desmoglein 3 (dsg 3) IHC and to correlate the same with autoantibody levels and clinical severity in patients with PV and PF.
METHODS
Forty-nine clinically, histologically, and DIF-confirmed cases of pemphigus were included in the study. The IHC patterns were scored from 0 to 3+ with 3+ dsg 1 IHC exhibiting intense membranous staining in the upper layers of the epidermis and 3+ dsg 3 IHC showing intense basal layer staining. Enzyme-linked immunosorbent assay (ELISA) for anti-dsg 1 and 3 antibodies was performed in 38 cases where serum samples were available. The pemphigus disease activity index system was utilized for clinical scoring.
RESULTS
A 0 to 1+ score was observed for dsg 1 IHC in 100% of PF cases. A score of 0 to 1+ was observed for dsg 3 IHC in 97.3% of PV cases. One hundred percent of cases with PF and 83.9% of patients with PV tested positive for ELISA anti-dsg 1 and 3 antibody titers, respectively. Anti-dsg 1 and 3 ELISA titers significantly correlated with the dsg 1 and dsg 3 IHC scores. The mucosal scores showed a significant association with both dsg 1 and 3 IHC (p < 0.001). The cutaneous scores showed a significant association with the dsg 3 IHC (p < 0.001).
CONCLUSION
The IHC patterns for dsg 1 and 3 proved reliable in giving concordant results with the ELISA antibody titers and clinical severity.
背景
天疱疮是一种慢性、潜在致命的自身免疫性大疱性疾病。寻常型天疱疮（PV）和落叶型天疱疮（PF）是两种常见的亚型。PV 是最常见且侵袭性最强的类型，其特征为口腔黏膜糜烂和皮肤损伤。PF 表现为头皮、面部和上躯干水疱，且黏膜不受累。直接免疫荧光（DIF）是诊断的金标准。免疫组织化学（IHC）是一种新兴的替代诊断工具。在这项研究中，我们的目的是确定 IHC 检测 desmoglein 1（dsg1）和 desmoglein 3（dsg3）的染色模式，并将其与 PV 和 PF 患者的自身抗体水平和临床严重程度相关联。
方法
本研究纳入了 49 例经临床、组织学和 DIF 确诊的天疱疮患者。IHC 模式评分范围为 0 至 3+，3+的 dsg1 IHC 表现为表皮上层强烈的膜状染色，3+的 dsg3 IHC 显示强烈的基底细胞染色。对 38 例有血清样本的患者进行了酶联免疫吸附试验（ELISA）检测抗 dsg1 和 3 抗体。采用天疱疮疾病活动指数系统进行临床评分。
结果
PF 病例的 dsg1 IHC 评分均为 0 至 1+。PV 病例的 dsg3 IHC 评分均为 0 至 1+。PF 病例的 100%和 PV 病例的 83.9%检测到 ELISA 抗 dsg1 和 3 抗体滴度阳性。ELISA 抗 dsg1 和 3 抗体滴度与 dsg1 和 dsg3 IHC 评分显著相关。黏膜评分与 dsg1 和 dsg3 IHC 均有显著相关性（p<0.001）。皮肤评分与 dsg3 IHC 有显著相关性（p<0.001）。
结论
dsg1 和 dsg3 IHC 模式在与 ELISA 抗体滴度和临床严重程度一致方面是可靠的。
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