Hosoda Satomi, Adachi Akimasa, Suzuki Masayuki, Yamada Tomoko, Komine Mayumi, Murata Satoru, Ohtsuki Mamitaro
Department of Dermatology, Jichi Medical University, Shimotsuke, Japan.
J Dermatol. 2016 Feb;43(2):194-6. doi: 10.1111/1346-8138.13041. Epub 2015 Jul 29.
We report a case involving a 62-year-old woman with in vivo-bound immunoglobulin (Ig)G and IgA antibodies in both the intercellular space (ICS) and basement membrane zone (BMZ). Her clinical and histopathological features were identical with those of pemphigus vulgaris, while the immunopathological findings suggested IgG/IgA pemphigus. Direct immunofluorescence (IF) showed in vivo-bound IgG and IgA antibodies in the ICS and BMZ, whereas indirect IF showed circulating IgG but not IgA antibodies in the ICS and BMZ. The anti-ICS IgG bound to desmoglein-3, while the anti-BMZ antibodies bound to the epidermal side of 1 mol/L NaCl-split skin. To the best of our knowledge, only two similar cases have been reported so far. Furthermore, we also examined IgG subclass distribution of the in vivo-bound and circulating anti-ICS and BMZ antibodies, and found that IgG1, IgG2 and IgG4 bound to ICS of the lesional skins, while IgG1 and IgG3 bound to the BMZ. The circulating anti-ICS antibodies belonged to IgG1 and IgG4, while the circulating anti-BMZ antibodies to IgG1, IgG2 and IgG4. We report a case involving a 62-year-old woman with in vivo-bound immunoglobulin (Ig)G and IgA antibodies in both the intercellular space (ICS) and basement membrane zone (BMZ). Her clinical and histopathological features were identical with those of pemphigus vulgaris, while the immunopathological findings suggested IgG/IgA pemphigus. Direct immunofluorescence (IF) showed in vivo-bound IgG and IgA antibodies in the ICS and BMZ, whereas indirect IF showed circulating IgG but not IgA antibodies in the ICS and BMZ. The anti-ICS IgG bound to desmoglein-3, while the anti-BMZ antibodies bound to the epidermal side of 1 mol/L NaCl-split skin. To the best of our knowledge, only two similar cases have been reported so far. Furthermore, we also examined IgG subclass distribution of the in vivo-bound and circulating anti-ICS and BMZ antibodies, and found that IgG1, IgG2 and IgG4 bound to ICS of the lesional skins, while IgG1 and IgG3 bound to the BMZ. The circulating anti-ICS antibodies belonged to IgG1 and IgG4, while the circulating anti-BMZ antibodies to IgG1, IgG2 and IgG4.
我们报告了一例62岁女性病例，其细胞间空间（ICS）和基底膜带（BMZ）均存在体内结合的免疫球蛋白（Ig）G和IgA抗体。她的临床和组织病理学特征与寻常型天疱疮相同，而免疫病理学结果提示为IgG/IgA天疱疮。直接免疫荧光（IF）显示ICS和BMZ中有体内结合的IgG和IgA抗体，而间接IF显示ICS和BMZ中有循环IgG抗体但无IgA抗体。抗ICS IgG与桥粒芯糖蛋白-3结合，而抗BMZ抗体与1mol/L NaCl分离皮肤的表皮侧结合。据我们所知，迄今为止仅报道过两例类似病例。此外，我们还检测了体内结合的和循环的抗ICS及BMZ抗体的IgG亚类分布，发现IgG1、IgG2和IgG4与皮损的ICS结合，而IgG1和IgG3与BMZ结合。循环抗ICS抗体属于IgG1和IgG4，而循环抗BMZ抗体属于IgG1、IgG2和IgG4。我们报告了一例62岁女性病例，其细胞间空间（ICS）和基底膜带（BMZ）均存在体内结合的免疫球蛋白（Ig）G和IgA抗体。她的临床和组织病理学特征与寻常型天疱疮相同，而免疫病理学结果提示为IgG/IgA天疱疮。直接免疫荧光（IF）显示ICS和BMZ中有体内结合的IgG和IgA抗体，而间接IF显示ICS和BMZ中有循环IgG抗体但无IgA抗体。抗ICS IgG与桥粒芯糖蛋白-3结合，而抗BMZ抗体与1mol/L NaCl分离皮肤的表皮侧结合。据我们所知，迄今为止仅报道过两例类似病例。此外，我们还检测了体内结合的和循环的抗ICS及BMZ抗体的IgG亚类分布，发现IgG1、IgG2和IgG4与皮损的ICS结合，而IgG1和IgG3与BMZ结合。循环抗ICS抗体属于IgG1和IgG4，而循环抗BMZ抗体属于IgG1、IgG2和IgG4。
