自身免疫性表皮下大疱病诊疗共识（2022）

doi:10.35541/cjd.20210532

中华皮肤科杂志 ›› 2022, Vol. 55 ›› Issue (1): 1-11.doi: 10.35541/cjd.20210532

自身免疫性表皮下大疱病诊疗共识（2022）

中华医学会皮肤性病学分会中国医师协会皮肤科医师分会

中华医学会皮肤性病学分会中国医师协会皮肤科医师分会

收稿日期:2021-07-21 修回日期:2021-11-02 发布日期:2021-12-31
通讯作者: 王刚；晋红中 E-mail:xjwgang@fmmu.edu.cn

Consensus on the diagnosis and treatment of autoimmune subepidermal bullous diseases （2022）

Chinese Society of Dermatology, China Dermatologist Association

Chinese Society of Dermatology, China Dermatologist Association

Received:2021-07-21 Revised:2021-11-02 Published:2021-12-31
Contact: Wang Gang; Jin Hongzhong E-mail:xjwgang@fmmu.edu.cn

摘要/Abstract

摘要： 【摘要】自身免疫性表皮下大疱病主要包括大疱性类天疱疮、黏膜类天疱疮、瘢痕性类天疱疮、妊娠类天疱疮、扁平苔藓类天疱疮、线状IgA大疱性皮病、获得性大疱性表皮松解症、抗p200/层黏连蛋白γ1类天疱疮及疱疹样皮炎，不同的疾病靶抗原和致病性自身抗体存在差异，临床表现有相似之处也有明显不同，诊断与鉴别诊断有赖临床、病理、免疫病理检查及血清抗体检测等。为规范此类疾病的临床诊疗，中华医学会皮肤性病学分会和中国医师协会皮肤科医师分会组织本领域专家，依据近年国内外临床研究数据和指南共识，制订本共识。

关键词: 皮肤疾病, 水疱大疱性, 类天疱疮, 大疱性, 类天疱疮, 良性黏膜, 诊断, 治疗, 自身免疫性表皮下大疱病, 专家共识

Abstract: 【Abstract】 Autoimmune subepidermal bullous diseases mainly include bullous pemphigoid, mucous membrane pemphigoid, cicatricial pemphigoid, pemphigoid gestationis, lichen planus pemphigoides, linear IgA bullous dermatosis, epidermolysis bullosa acquisita, anti-p200/anti-laminin γ1 pemphigoid and dermatitis herpetiformis. Target antigens and pathogenic autoantibodies vary substantially among the above disorders, and there were similarities and differences in their clinical manifestations. Diagnosis and differential diagnosis commonly rely on clinical manifestations, pathological, immunopathological examinations and serum antibody testing. In order to standardize the management of autoimmune subepidermal bullous diseases, Chinese Society of Dermatology and China Dermatologist Association organized experts in this field to develop this consensus based on recent Chinese and international clinical research data, guidelines and consensus.

Key words: Skin diseases, vesiculobullous, Pemphigoid, bullous, Pemphigoid, benign mucous membrane, Diagnosis, Therapy, Autoimmune subepidermal bullous diseases, Expert consensus

中华医学会皮肤性病学分会中国医师协会皮肤科医师分会. 自身免疫性表皮下大疱病诊疗共识（2022）[J]. 中华皮肤科杂志, 2022,55(1):1-11. doi:10.35541/cjd.20210532

Chinese Society of Dermatology, China Dermatologist Association. Consensus on the diagnosis and treatment of autoimmune subepidermal bullous diseases （2022）[J]. Chinese Journal of Dermatology, 2022, 55(1): 1-11.doi:10.35541/cjd.20210532

参考文献 33

［1］	Schmidt E, Groves RW. Rook′s textbook of dermatology［M］. 9th ed. 2016［2021⁃01⁃19］. https://onlinelibrary.wiley.com/doi/10.1002/9781118441213.rtd0051. doi: 10.1002/9781118441213. rtd0051.
［2］	Montagnon CM, Tolkachjov SN, Murrell DF, et al. Subepithelial autoimmune blistering dermatoses: clinical features and diagnosis［J］. J Am Acad Dermatol, 2021,85（1）:1⁃14. doi: 10. 1016/j.jaad.2020.11.076.
［3］	Schmidt E, Zillikens D. Pemphigoid diseases［J］. Lancet, 2013,381（9863）:320⁃332. doi: 10. 1016/S0140⁃6736（12）61140⁃4.
［4］	Persson M, Harman KE, Vinogradova Y, et al. Incidence, prevalence and mortality of bullous pemphigoid in England 1998-2017: a population⁃based cohort study［J］. Br J Dermatol, 2021,184（1）:68⁃77. doi: 10.1111/bjd.19022.
［5］	Santi CG, Gripp AC, Roselino AM, et al. Consensus on the treatment of autoimmune bullous dermatoses: bullous pemphigoid, mucous membrane pemphigoid and epidermolysis bullosa acquisita ⁃ Brazilian Society of Dermatology［J］. An Bras Dermatol, 2019,94（2 Suppl 1）:33⁃47. doi: 10.1590/abd1806⁃4841.2019940207.
［6］	Di Lernia V, Casanova DM, Goldust M, et al. Pemphigus vulgaris and bullous pemphigoid: update on diagnosis and treatment［J］. Dermatol Pract Concept, 2020,10（3）:e2020050. doi: 10.5826/dpc.1003a50.
［7］	Meijer JM, Diercks G, de Lang E, et al. Assessment of diagnostic strategy for early recognition of bullous and nonbullous variants of pemphigoid［J］. JAMA Dermatol, 2019,155（2）:158⁃165. doi: 10.1001/jamadermatol.2018.4390.
［8］	Yuan H, Pan M. Endoscopic characteristics of oesophagus involvement in mucous membrane pemphigoid［J］. Br J Dermatol, 2017,177（4）:902⁃903. doi: 10.1111/bjd.15804.
［9］	中国医师协会皮肤科医师分会自身免疫性疾病亚专业委员会. 大疱性类天疱疮诊断和治疗的专家建议［J］. 中华皮肤科杂志, 2016,49（6）:384⁃387. doi: 10.3760/cma.j.issn.0412⁃4030. 2016.06.003.
［10］	Fujio Y, Kojima K, Hashiguchi M, et al. Validation of chemiluminescent enzyme immunoassay in detection of autoantibodies in pemphigus and pemphigoid［J］. J Dermatol Sci, 2017,85（3）:208⁃215. doi: 10.1016/j.jdermsci.2016.12.007.
［11］	Murrell DF, Daniel BS, Joly P, et al. Definitions and outcome measures for bullous pemphigoid: recommendations by an international panel of experts［J］. J Am Acad Dermatol, 2012,66（3）:479⁃485. doi: 10.1016/j.jaad.2011.06.032.
［12］	Masmoudi W, Vaillant M, Vassileva S, et al. International validation of the bullous pemphigoid disease area index severity score and calculation of cut⁃off values for defining mild, moderate and severe types of bullous pemphigoid［J］. Br J Dermatol, 2021,184（6）:1106⁃1112. doi: 10.1111/bjd.19611.
［13］	王娣, 陈喜雪, 朱学骏. 124例大疱性类天疱疮治疗回顾［J］. 中华皮肤科杂志, 2007,40（1）:7⁃9. doi: 10.3760/j.issn:0412⁃4030.2007.01.005.
［14］	Schmidt E, Sticherling M, Sárdy M, et al. S2k guidelines for the treatment of pemphigus vulgaris/foliaceus and bullous pemphigoid: 2019 update［J］. J Dtsch Dermatol Ges, 2020,18（5）:516⁃526. doi: 10.1111/ddg.14097.
［15］	Patel PM, Jones VA, Murray TN, et al. A review comparing international guidelines for the management of bullous pemphigoid, pemphigoid gestationis, mucous membrane pemphigoid, and epidermolysis bullosa acquisita［J］. Am J Clin Dermatol, 2020,21（4）:557⁃565. doi: 10.1007/s40257⁃020⁃00513⁃3.
［16］	Ujiie H, Iwata H, Yamagami J, et al. Japanese guidelines for the management of pemphigoid （including epidermolysis bullosa acquisita）［J］. J Dermatol, 2019,46（12）:1102⁃1135. doi: 10. 1111/1346⁃8138.15111.
［17］	Montagnon CM, Lehman JS, Murrell DF, et al. Subepithelial autoimmune bullous dermatoses disease activity assessment and therapy［J］. J Am Acad Dermatol, 2021,85（1）:18⁃27. doi: 10. 1016/j.jaad.2020.05.161.
［18］	Hattori Y, Takahashi T, Seishima M. Bullous pemphigoid successfully treated with a combination therapy of plasmapheresis followed by intravenous high dose immunoglobulin［J］. Ther Apher Dial, 2017,21（4）:421⁃423. doi: 10.1111/1744⁃9987.12536.
［19］	James T, Salman S, Stevenson B, et al. IgE blockade in autoimmunity: omalizumab induced remission of bullous pemphigoid［J］. Clin Immunol, 2019,198:54⁃56. doi: 10.1016/j.clim.2018.12.015.
［20］	Abdat R, Waldman RA, de Bedout V, et al. Dupilumab as a novel therapy for bullous pemphigoid: a multicenter case series［J］. J Am Acad Dermatol, 2020,83（1）:46⁃52. doi: 10.1016/j.jaad.2020.01.089.
［21］	Murrell DF, Marinovic B, Caux F, et al. Definitions and outcome measures for mucous membrane pemphigoid: recommendations of an international panel of experts［J］. J Am Acad Dermatol, 2015,72（1）:168⁃174. doi: 10.1016/j.jaad.2014.08.024.
［22］	Lytvyn Y, Rahat S, Mufti A, et al. Biologic treatment outcomes in mucous membrane pemphigoid: a systematic review［J］. J Am Acad Dermatol, 2021［2021⁃02⁃04］. https://www.jaad.org/article/S0190⁃9622（21）00010⁃4/fulltext. doi: 10.1016/j.jaad.2020.12. 056.
［23］	Fong M, Gandhi GR, Gharbi A, et al. Pemphigoid gestationis［M/OL］//StatPearls ［Internet］. Treasure Island （FL）: StatPearls Publishing, 2021［2021⁃02⁃05］.
［24］	Genovese G, Derlino F, Cerri A, et al. A systematic review of treatment options and clinical outcomes in pemphigoid gestationis［J］. Front Med （Lausanne）, 2020,7:604945. doi: 10.3389/fmed.2020.604945.
［25］	Kwon CW, Murthy RK, Kudchadkar R, et al. Pembrolizumab⁃induced lichen planus pemphigoides in a patient with metastatic Merkel cell carcinoma［J］. JAAD Case Rep, 2020,6（10）:1045⁃1047. doi: 10.1016/j.jdcr.2020.03.007.
［26］	Sugawara A, Koga H, Abe T, et al. Lichen planus⁃like lesion preceding bullous pemphigoid development after programmed cell death protein⁃1 inhibitor treatment［J］. J Dermatol, 2021,48（3）:401⁃404. doi: 10.1111/1346⁃8138.15693.
［27］	Lim A, Tang PY, Oh CC. Lichen planus pemphigoides after body tattooing［J］. J Cosmet Dermatol, 2020,19（11）:3048⁃3052. doi: 10.1111/jocd.13553.
［28］	Holtsche MM, Goletz S, von Georg A, et al. Serologic characterization of anti⁃p200 pemphigoid: epitope spreading as a common phenomenon［J］. J Am Acad Dermatol, 2021,84（4）:1155⁃1157. doi: 10.1016/j.jaad.2020.07.076.
［29］	Sun Y, Lin Y, Yang B, et al. The HLA alleles B0801 and DRB10301 are associated with dermatitis herpetiformis in a Chinese population［J］. J Invest Dermatol, 2016,136（2）:530⁃532. doi: 10.1016/j.jid.2015.10.057.
［30］	Lorand L, Iismaa SE. Transglutaminase diseases: from biochemistry to the bedside［J］. FASEB J, 2019,33（1）:3⁃12. doi: 10.1096/fj.201801544R.
［31］	Abadie V, Kim SM, Lejeune T, et al. IL⁃15, gluten and HLA⁃DQ8 drive tissue destruction in coeliac disease［J］. Nature, 2020,578（7796）:600⁃604. doi: 10.1038/s41586⁃020⁃2003⁃8.
［32］	夏倩倩, 孙勇虎, 张福仁. 中国疱疹样皮炎特征分析：1958—2018年文献回顾［J］. 中华皮肤科杂志, 2020,53（11）:928⁃931. doi: 10.35541/cjd.20191076.
［33］	Reunala T, Hervonen K, Salmi T. Dermatitis herpetiformis: an update on diagnosis and management［J］. Am J Clin Dermatol, 2021,22（3）:329⁃338. doi: 10.1007/s40257⁃020⁃00584⁃2.

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自身免疫性表皮下大疱病诊疗共识（2022）

Consensus on the diagnosis and treatment of autoimmune subepidermal bullous diseases （2022）

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