Kamiya Koji, Aoyama Yumi, Wakashima Chie, Kudo Tomoko, Nakajima Kimiko, Sano Shigetoshi, Ishii Norito, Teye Kwesi, Hashimoto Takashi, Iwatsuki Keiji, Tokura Yoshiki
Department of Dermatology, Hamamatsu University School of Medicine, Hamamatsu, Japan.
Department of Dermatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.
J Dermatol. 2016 Apr;43(4):429-31. doi: 10.1111/1346-8138.13086. Epub 2015 Sep 2.
In patients with pemphigus vulgaris (PV), pathogenic immunoglobulin (Ig)G antibodies are most commonly directed against desmoglein 3 (Dsg3). It has recently been reported, however, that IgG anti-desmocollin 3 (Dsc3) antibodies are detected in some cases of pemphigus with or without IgG anti-Dsg3 antibodies. We present a case of pemphigus with IgG antibodies against Dsg3 and Dsc3. Subsequent studies showed that the cell surface distribution pattern of Dsc3 but not Dsg3 was altered, suggesting that suprabasal acantholytic blisters were induced by IgG anti-Dsc3 antibodies rather than IgG anti-Dsg3 antibodies. Our case suggests that anti-Dsc3 antibodies may be pathogenic in cases positive for the dual cadherin autoantibodies.
在寻常型天疱疮（PV）患者中，致病性免疫球蛋白（Ig）G抗体最常针对桥粒芯糖蛋白3（Dsg3）。然而，最近有报道称，在一些有或没有IgG抗Dsg3抗体的天疱疮病例中检测到IgG抗桥粒芯胶蛋白3（Dsc3）抗体。我们报告了1例同时具有抗Dsg3和抗Dsc3 IgG抗体的天疱疮病例。随后的研究表明，Dsc3而非Dsg3的细胞表面分布模式发生了改变，提示棘层松解水疱是由IgG抗Dsc3抗体而非IgG抗Dsg3抗体诱导产生的。我们的病例表明，在双钙黏蛋白自身抗体阳性的病例中，抗Dsc3抗体可能具有致病性。
