PDF(Pubmed)
Abstract：
BACKGROUND: Anti-desmoglein (Dsg)1 is produced in pemphigus foliaceus (PF), affecting exclusively the skin. Pemphigus vulgaris (PV) shows the production of anti-Dsg3 in the mucosal form, and anti-Dsg1 and 3 in the mucocutaneous form. Anti-Dsg3 autoantibodies have been rarely reported in PF.
OBJECTIVE: To determine the factors associated with the production and pathogenicity of anti-Dsg3 in PF.
METHODS: Comparative analytical study of three patients groups: 16 PF-anti-Dsg3+, and 42 PF-anti-Dsg3(-) and 22 PV treatment-naïve cases. Serum was used in the anti-Dsg1 and 3 ELISA, and in immunoblotting (IB) with human epidermis extract. The expression of Dsg1 and 3 in paraffin sections was analyzed by immunohistochemistry (IHC). HLA-DRB1 alleles were compiled from a database.
RESULTS: In the PF-anti-Dsg3+ group: age range similar to that of the PV group (p > 0.9999); predominance of the generalized form of PF (p = 0.002); anti-Dsg3 titers lower than those of PV (p < 0.0001); IB confirmed Dsg3 identification in one (8.33%) of 12 patients; IHC showed exclusive cytoplasmic internalization of Dsg1; HLA-DRB1 alleles of susceptibility to PF, with the absence of alleles associated with PV, in the five typed patients.
CONCLUSIONS: Most of the patients in the PF-anti-Dsg3+ group were undergoing treatment.
CONCLUSIONS: The presence of anti-Dsg3 antibodies in PF was related to older age (comparable to that of PV) and the generalized form of PF. The non-pathogenicity of anti-Dsg3 antibodies in PF can be attributed to the low serum anti-Dsg3 titers, the lack of Dsg3 internalization as detected by IHC, and the absence of PV-associated HLA-DRB1 alleles.
OBJECTIVE: To determine the factors associated with the production and pathogenicity of anti-Dsg3 in PF.
METHODS: Comparative analytical study of three patients groups: 16 PF-anti-Dsg3+, and 42 PF-anti-Dsg3(-) and 22 PV treatment-naïve cases. Serum was used in the anti-Dsg1 and 3 ELISA, and in immunoblotting (IB) with human epidermis extract. The expression of Dsg1 and 3 in paraffin sections was analyzed by immunohistochemistry (IHC). HLA-DRB1 alleles were compiled from a database.
RESULTS: In the PF-anti-Dsg3+ group: age range similar to that of the PV group (p > 0.9999); predominance of the generalized form of PF (p = 0.002); anti-Dsg3 titers lower than those of PV (p < 0.0001); IB confirmed Dsg3 identification in one (8.33%) of 12 patients; IHC showed exclusive cytoplasmic internalization of Dsg1; HLA-DRB1 alleles of susceptibility to PF, with the absence of alleles associated with PV, in the five typed patients.
CONCLUSIONS: Most of the patients in the PF-anti-Dsg3+ group were undergoing treatment.
CONCLUSIONS: The presence of anti-Dsg3 antibodies in PF was related to older age (comparable to that of PV) and the generalized form of PF. The non-pathogenicity of anti-Dsg3 antibodies in PF can be attributed to the low serum anti-Dsg3 titers, the lack of Dsg3 internalization as detected by IHC, and the absence of PV-associated HLA-DRB1 alleles.
摘要：
背景：抗桥粒蛋白（Dsg）1在天疱疮（PF）中产生，只影响皮肤。寻常型天疱疮（PV）显示粘膜形式的抗Dsg3的产生，和粘膜皮肤形式的抗Dsg1和3。抗Dsg3自身抗体在PF中很少报道。
目的：确定与PF中抗Dsg3的产生和致病性相关的因素。
方法：三个患者组的比较分析研究：16PF-抗Dsg3+，42例PF-抗Dsg3（-）和22例PV治疗初治病例。血清用于抗Dsg1和3ELISA，和用人表皮提取物进行免疫印迹(IB)。通过免疫组织化学(IHC)分析石蜡切片中Dsg1和3的表达。从数据库编辑HLA-DRB1等位基因。
结果：在PF-抗Dsg3组中：年龄范围与PV组相似（p>0.9999）；PF的广泛性形式占优势（p=0.002）；抗Dsg3滴度低于PV（p<0.0001）；IB证实了12例患者的一个（8.33％）中的Dsg3鉴定；HLA内在化的IHCB1显示对细胞的特异性易感性由于缺乏与PV相关的等位基因，在五个打字的病人中。
结论：PF-抗Dsg3+组的大部分患者正在接受治疗。
结论：PF中抗Dsg3抗体的存在与年龄（与PV相当）和PF的普遍形式有关。抗Dsg3抗体在PF中的非致病性可归因于低血清抗Dsg3滴度，IHC检测到缺乏Dsg3内化，和缺乏PV相关的HLA-DRB1等位基因。
目的：确定与PF中抗Dsg3的产生和致病性相关的因素。
方法：三个患者组的比较分析研究：16PF-抗Dsg3+，42例PF-抗Dsg3（-）和22例PV治疗初治病例。血清用于抗Dsg1和3ELISA，和用人表皮提取物进行免疫印迹(IB)。通过免疫组织化学(IHC)分析石蜡切片中Dsg1和3的表达。从数据库编辑HLA-DRB1等位基因。
结果：在PF-抗Dsg3组中：年龄范围与PV组相似（p>0.9999）；PF的广泛性形式占优势（p=0.002）；抗Dsg3滴度低于PV（p<0.0001）；IB证实了12例患者的一个（8.33％）中的Dsg3鉴定；HLA内在化的IHCB1显示对细胞的特异性易感性由于缺乏与PV相关的等位基因，在五个打字的病人中。
结论：PF-抗Dsg3+组的大部分患者正在接受治疗。
结论：PF中抗Dsg3抗体的存在与年龄（与PV相当）和PF的普遍形式有关。抗Dsg3抗体在PF中的非致病性可归因于低血清抗Dsg3滴度，IHC检测到缺乏Dsg3内化，和缺乏PV相关的HLA-DRB1等位基因。
