中华皮肤科杂志 ›› 2023, Vol. 56 ›› Issue (9): 832-838.doi: 10.35541/cjd.20230132

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表现为环状红斑水疱的自身免疫性表皮下水疱病25例回顾性分析

荆可1    王媛1    李锁1    冯素英2   

  1. 1中国医学科学院  北京协和医学院皮肤病医院皮肤内科，南京  210042；2中国医学科学院  北京协和医学院皮肤病医院性病科，南京  210042
    王媛现在昆明医科大学第二附属医院皮肤性病科，昆明  650101

  • 收稿日期:2023-03-09 修回日期:2023-06-28 发布日期:2023-09-07
  • 通讯作者: 冯素英 E-mail:fengsy@pumcderm.cams.cn
  • 基金资助:
    江苏省卫生健康委科研项目（ZD2021035）

Autoimmune subepidermal bullous diseases characterized by annular erythema and blisters: a retrospective analysis of 25 cases

Jing Ke1, Wang Yuan1, Li Suo1, Feng Suying2   

  1. 1Department of Dermatology, Hospital of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing 210042, China; 2Department of Venereology, Hospital of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing 210042, China
    Wang Yuan is now working at the Department of Dermatology and Venereology, The Second Affiliated Hospital of Kunming Medical University, Kunming 650101, China

  • Received:2023-03-09 Revised:2023-06-28 Published:2023-09-07
  • Contact: Feng Suying E-mail:fengsy@pumcderm.cams.cn
  • Supported by:
    Scientific Research Project of Jiangsu Provincial Health Commission（ZD2021035）

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摘要： 【摘要】 目的 总结表现为环状红斑水疱的自身免疫性表皮下水疱病的临床、组织病理、免疫血清学及治疗特点。方法 回顾性分析2015—2022年就诊于中国医学科学院皮肤病医院表现为环状红斑水疱的自身免疫性表皮下水疱病患者的资料。结果 共纳入患者25例，男10例、女15例，年龄（39.21 ± 24.65）岁，包括线状IgA大疱性皮病9例，大疱性类天疱疮7例，抗P200类天疱疮5例，获得性大疱性表皮松解症4例，20例（80%）有不同程度瘙痒。15例（60%）出现真皮组织嗜酸性粒细胞浸润，11例（44%）外周血嗜酸性粒细胞计数增加，7例（28%）同时有嗜酸性粒细胞组织浸润和外周血嗜酸性粒细胞升高。盐裂皮肤-间接免疫荧光及免疫印迹实验显示，9例同时存在抗基底膜带IgG及IgA抗体，包括4例大疱性类天疱疮、1例线状IgA大疱性皮病、2例抗P200类天疱疮、2例获得性大疱性表皮松解症；5例同时存在多种抗基底膜带靶抗原的抗体。7例大疱性类天疱疮均予系统糖皮质激素治疗，其中5例联合免疫抑制剂，2例联合米诺环素；线状IgA大疱性皮病、抗P200类天疱疮、获得性大疱性表皮松解症患者对抗炎药物及氨苯砜治疗敏感。结论 多种类型自身免疫性表皮下水疱病均可有环状红斑、水疱表现，需根据血清学检查结果鉴别诊断。

关键词: 皮肤疾病, 水疱大疱性, 自身免疫疾病, 嗜酸细胞, 免疫球蛋白A, 环状皮损

Abstract: 【Abstract】 Objective To summarize clinical, histopathological, immunoserological, and therapeutic features of patients with autoimmune subepidermal bullous diseases characterized by annular erythema and blisters. Methods Clinical data were collected from patients with autoimmune subepidermal bullous diseases characterized by annular erythema and blisters in the Hospital of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College from 2015 to 2022, and their clinical, pathological, immunoserological and therapeutic characteristics were retrospectively analyzed. Results A total of 25 patients were enrolled, including 10 males and 15 females, aged 39.21 ± 24.65 years; there were 9 patients with linear IgA bullous dermatosis （LABD）, 7 with bullous pemphigoid （BP）, 5 with anti-P200 pemphigoid and 4 with epidermolysis bullosa acquisita （EBA）, and 20（80%） patients suffered from pruritus to different extents. Dermal eosinophil infiltration was observed in 15 （60%） patients, elevated peripheral blood eosinophil counts in 11（44%）, and both were observed in 7（28%）. Indirect immunofluorescence on salt-split skin and Western blot analysis showed that both IgG and IgA anti-basement membrane zone antibodies were present in 9 patients, including 4 with BP, 1 with LABD, 2 with anti-P200 pemphigoid, and 2 with EBA; and multiple antibodies against different basement membrane zone antigens were present in 5. All the 7 BP patients were treated with systemic glucocorticoids, including 5 （71.4%） receiving combined immunosuppressive agents, and 2 receiving combined minocycline; the patients with LABD, anti-P200 pemphigoid or EBA were sensitive to anti-inflammatory drugs and dapsone. Conclusion Multiple types of autoimmune subepidermal bullous diseases may manifest as annular erythema and blisters, and it is necessary to make a differential diagnosis based on immunoserological tests.

Key words: Skin diseases, vesiculobullous, Autoimmune diseases, Eosinophils, Immunoglobulin A, Annular lesions

引用本文

荆可 王媛 李锁 冯素英. 表现为环状红斑水疱的自身免疫性表皮下水疱病25例回顾性分析[J]. 中华皮肤科杂志, 2023,56(9):832-838. doi:10.35541/cjd.20230132

Jing Ke, Wang Yuan, Li Suo, Feng Suying. Autoimmune subepidermal bullous diseases characterized by annular erythema and blisters: a retrospective analysis of 25 cases[J]. Chinese Journal of Dermatology, 2023, 56(9): 832-838.doi:10.35541/cjd.20230132