免疫球蛋白和补体的免疫组织化学染色可以帮助诊断临床和组织学发现与自身免疫性大疱性皮肤病(AIBD)一致的患者。我们旨在研究免疫组织化学标志物在AIBD病灶活检和病灶周围冷冻样本中的诊断价值。我们纳入了136例病例,其中在2019年1月至2023年1月之间收集了用于直接免疫荧光(DIF)检查的病灶活检和病灶周围样本,并初步诊断为AIBD。所有诊断都通过评估临床来重新确认,组织病理学,和血清学发现和DIF结果(C3,IgG,IgA,或与临床诊断相容的IgM阳性),尽管DIF结果被认为是优先考虑的。确诊后,样本被归类为AIBD或其他。将与皮肤活检同时获得的DIF周围组织解冻,并在-80°C下储存。和制备FFPE组织。我们进行了免疫组织化学染色(C4d,C3d,IgG,和IgG4)在病灶和病灶周围样品的FFPE组织上。坚强,线性,真皮表皮交界处或表皮内起泡空间的颗粒染色模式被认为是阳性,符合病例的诊断。将除AIBD以外的病例用作阴性对照组织以评估免疫组织化学标记物的特异性。在136个案例中,52例确诊为AIBD。在病变样本中,C4d的灵敏度,C3d,IgG,IgG4为80.6%,69.4%,75%,5.7%,相应的特异性为100%,98.7%,89.6%,和97.4%,分别在类天疱疮疾病中的敏感度为18.2%,9.1%,70%,和9.1%和98.7%的特异性,100%,89.6%,和97.4%,分别在天疱疮疾病中。在冷冻样本中,我们在有限数量的病例中检测到表达。C4d的灵敏度,C3d,IgG,IgG4为8.7%,2.2%,19.4%,和2.2%,相应的特异性为100%,100%,98.5%,分别为98.6。病灶组织和病灶周围冷冻样品的IHC结果之间无至轻微的一致率。C4d的Kappa系数,C3d,IgG,IgG4为0.120(P=0.029),0.111(P=0.050),0.203(P=0.003),和-0.15(P=0.846),分别。C4d免疫组织化学染色,C3d,IgG,从病灶收集的活检样本上的IgG4可以指导AIBD的诊断,从而消除了额外的活检和加速诊断过程的需要。
Immunohistochemical staining with immunoglobulins and complements may aid the diagnosis of patients whose clinical and histological findings are consistent with autoimmune bullous dermatoses (AIBD). We aimed to investigate the diagnostic value of immunohistochemical markers in lesional biopsy and perilesional frozen samples in AIBD. We included 136 cases from whom lesional biopsies and perilesional samples for direct immunofluorescence (DIF) examination were collected with a preliminary diagnosis of AIBD between January 2019 and January 2023. All diagnoses were reconfirmed by evaluating the clinical, histopathological, and serological findings and DIF results (C3, IgG, IgA, or IgM positivity compatible with the clinical diagnosis) altogether, although DIF results were considered a priority. After confirming the diagnoses, the samples were categorized as AIBD or the others. The perilesional tissues obtained for DIF simultaneously with skin biopsy and stored at -80 °C were thawed, and FFPE tissues were prepared. We performed immunohistochemical staining (C4d, C3d, IgG, and
IgG4) on FFPE tissues of both lesional and perilesional samples. Strong, linear, or granular staining patterns at the dermoepidermal junction or the intraepidermal blistering space were considered positive in line with the diagnosis of the case. Cases other than AIBD were used as negative control tissues to assess the specificity of immunohistochemical markers. Of the 136 cases, 52 were diagnosed with AIBD. In lesional samples, the sensitivity of C4d, C3d, IgG, and
IgG4 was 80.6 %, 69.4 %, 75 %, and 5.7 % with corresponding specificity of 100 %, 98.7 %, 89.6 %, and 97.4 %, respectively in pemphigoid diseases compared to a sensitivity of 18.2 %, 9.1 %, 70 %, and 9.1 % and specificity of 98.7 %, 100 %, 89.6 %, and 97.4 %, respectively in pemphigus diseases. In frozen samples, we detected expression in a limited number of cases. The sensitivity of C4d, C3d, IgG, and
IgG4 was 8.7 %, 2.2 %, 19.4 %, and 2.2 %, with corresponding specificity of 100 %, 100 %, 98.5 %, and 98.6, respectively. There was a none to slight concordance rate between the IHC results of lesional tissues and perilesional frozen samples. Kappa coefficients for C4d, C3d, IgG, and
IgG4 were 0.120 (P = 0.029), 0.111 (P = 0.050), 0.203 (P = 0.003), and - 0.15 (P = 0.846), respectively. Immunohistochemical staining with C4d, C3d, IgG, and
IgG4 on biopsy samples collected from lesions may guide the diagnosis of AIBD, thereby eliminating the need for an additional biopsy and accelerating the diagnostic process.