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Abstract：
IgG4-RD is a multisystem fibroinflammatory disease characterized by the infiltration of tissues by IgG4 plasma cells. Combined skin and biliary tract involvement in IgG4-RD has not been described. We present perhaps the most comprehensive analysis of lymphocyte subsets in the first case of IgG4-related generalized skin rash and first case of combined skin and biliary tract manifestations. A 55-year-old male presented with painful jaundice and generalized macular pigmented pruritic eruptions, and CT abdomen revealed biliary obstruction. Ampulla and skin biopsies were subjected to histology and immunostaining. Naïve, central memory (TCM), effector memory (TEM), terminally differentiated effector memory (TEMRA) subsets of CD4+ and CD8+ T cells, T follicular helper subsets, naïve, transitional, marginal zone (MZ), germinal center (GC), IgM memory, and class-switched memory (CSM) B cells, and T follicular regulatory, regulatory B cells, CD4 Treg, and CD8 Treg were analyzed. Serum IgG4 was elevated at 448 mg/dL. Ampula biopsy showed lamina propria fibrosis and increased IgG4-positive plasma cells. Skin punch biopsy showed lymphoplasmacytic infiltrates with a 67% ratio of IgG4+:IgG+ plasma cells. CD4+TN and CD4+TCM decreased, whereas CD4+TEM increased. Naïve B cells increased; transitional, MZ, CSM, GC B cells, and plasmablasts decreased compared to control. CD4 Treg increased, whereas CD8 Treg and Breg decreased. In conclusion, IgG-RD may present with combined biliary tract and generalized dermatological manifestations. Changes in regulatory lymphocytes suggest their role in the pathogenesis of IgG4-RD.
摘要：
IgG4-RD是一种多系统纤维炎性疾病，其特征是IgG4浆细胞浸润组织。尚未描述IgG4-RD的皮肤和胆道联合受累。我们可能对首例IgG4相关的全身性皮疹和首例皮肤和胆道联合表现的淋巴细胞亚群进行了最全面的分析。一名55岁的男性表现为疼痛性黄疸和泛发性黄斑色素性瘙痒，腹部CT显示胆道梗阻。对壶腹和皮肤活检进行组织学和免疫染色。天真,中央存储器(TCM),效应记忆(TEM),CD4+和CD8+T细胞的终末分化效应记忆（TEMRA）亚群，T卵泡辅助亚群，天真,过渡,边缘区域（MZ），生发中心（GC），IgM记忆,和类别交换存储器(CSM)B单元，和T卵泡调节，调节性B细胞,CD4Treg,和CD8Treg进行分析。血清IgG4升高至448mg/dL。壶腹活检显示固有层纤维化和IgG4阳性浆细胞增加。皮肤穿刺活检显示淋巴浆细胞浸润，IgG4：IgG浆细胞比例为67％。CD4+TN和CD4+中药降低，而CD4+TEM升高。原初B细胞增加；过渡性，MZ,CSM,GCB细胞，与对照组相比，浆细胞减少。CD4Treg升高,而CD8Treg和Breg下降。总之,IgG-RD可能表现为合并的胆道和全身性皮肤病学表现。调节性淋巴细胞的变化表明它们在IgG4-RD的发病机理中的作用。
