【开放获取】 急危重皮肤病的诊疗原则、现状、挑战和展望

doi:10.35541/cjd.20240527

Abstract

Abstract: 【Abstract】 Critical dermatological emergencies generally refer to a group of dermatoses characterized by urgent onset, rapid progression, and potential life-threatening complications. This commentary elucidates the clinical characteristics of, diagnostic procedures, and therapeutic strategies for critical dermatological emergencies, including severe cutaneous adverse reactions, autoimmune bullous diseases, and psoriasis, emphasizing the importance of early recognition, diagnosis, standardized treatment, and multidisciplinary collaboration in their management. Furthermore, this commentary provides an in-depth analysis of the current challenges and risks in managing these conditions, and proposes specific improvement measures, such as strengthening primary prevention, optimizing referral systems, and standardizing diagnostic and therapeutic procedures, aiming to provide practical clinical guidance for the management of critical dermatological emergencies.

Key words: Critical illness, Critical dermatological emergencies, Drug eruptions, Skin diseases, vesiculobullous, Generalized pustular psoriasis, Erythrodermic psoriasis, Multidisciplinary management

He Chunxia, Jin Hongzhong . Principles, current status, challenges and perspectives in the diagnosis and treatment of critical dermatological emergencies[J]. Chinese Journal of Dermatology, 2025, 58(4): 307-314.doi:10.35541/cjd.20240527

References ［55］

［1］	Billings SD. Common and critical inflammatory dermatoses every pathologist should know［J］. Mod Pathol, 2020,33（Suppl 1）:107⁃117. doi: 10.1038/s41379⁃019⁃0400⁃z.
［2］	Freiman A, Borsuk D, Sasseville D. Dermatologic emergencies［J］. CMAJ, 2005,173（11）:1317⁃1319. doi: 10.1503/cmaj.050783.
［3］	Bromley M, Marsh S, Layton A. Life⁃threatening skin conditions presenting to critical care［J］. BJA Educ, 2021,21（10）:376⁃383. doi: 10.1016/j.bjae.2021.05.007.
［4］	Yang JJ, Maloney NJ, Bach DQ, et al. Dermatology in the emergency department: prescriptions, rates of inpatient admission, and predictors of high utilization in the United States from 1996 to 2012［J］. J Am Acad Dermatol, 2021,84（5）:1480⁃1483. doi: 10.1016/j.jaad.2020.07.055.
［5］	Isnard C, Ingen⁃Housz⁃Oro S, Fardet L, et al. Dermatological emergencies: evolution from 2008 to 2014 and perspectives［J］. J Eur Acad Dermatol Venereol, 2017,31（2）:274⁃279. doi: 10. 1111/jdv.13860.
［6］	Cazzaniga S, Heidemeyer K, Zahn CA, et al. Dermatological emergencies and determinants of hospitalization in Switzerland: a retrospective study［J］. J Eur Acad Dermatol Venereol, 2024. doi: 10.1111/jdv.20176.
［7］	Bancalari⁃Díaz D, Gimeno⁃Mateos LI, Cañueto J, et al. Dermatologic emergencies in a tertiary hospital: a descriptive study［J］. Actas Dermosifiliogr, 2016,107（8）:666⁃673. doi: 10.1016/j.ad.2016.05.001.
［8］	Hung SI, Mockenhaupt M, Blumenthal KG, et al. Severe cutaneous adverse reactions［J］. Nat Rev Dis Primers, 2024,10（1）:30. doi: 10.1038/s41572⁃024⁃00514⁃0.
［9］	Wei BM, Fox LP, Kaffenberger BH, et al. Drug⁃induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms. Part I. Epidemiology, pathogenesis, clinicopathological features, and prognosis［J］. J Am Acad Dermatol, 2024,90（5）:885⁃908. doi: 10.1016/j.jaad.2023.02.072.
［10］	Egami S, Yamagami J, Amagai M. Autoimmune bullous skin diseases, pemphigus and pemphigoid［J］. J Allergy Clin Immunol, 2020,145（4）:1031⁃1047. doi: 10.1016/j.jaci.2020.02.013.
［11］	Powers CM, Thakker S, Gulati N, et al. Bullous pemphigoid: a practical approach to diagnosis and management in the modern era［J］. J Am Acad Dermatol, 2025:S0190⁃9622（25）00185⁃00189 ［pii］. doi: 10.1016/j.jaad.2025.01.086.
［12］	Schmidt E, Kasperkiewicz M, Joly P. Pemphigus［J］. Lancet, 2019,394（10201）:882⁃894. doi: 10.1016/S0140⁃6736（19）31778⁃7.
［13］	Armstrong AW, Elston CA, Elewski BE, et al. Generalized pustular psoriasis: a consensus statement from the National Psoriasis Foundation［J］. J Am Acad Dermatol, 2024,90（4）:727⁃730. doi: 10.1016/j.jaad.2023.09.080.
［14］	Shao S, Wang G, Maverakis E, et al. Targeted treatment for erythrodermic psoriasis: rationale and recent advances［J］. Drugs, 2020,80（6）:525⁃534. doi: 10.1007/s40265⁃020⁃01283⁃2.
［15］	Prinz JC, Choon SE, Griffiths C, et al. Prevalence, comorbidities and mortality of generalized pustular psoriasis: a literature review［J］. J Eur Acad Dermatol Venereol, 2023,37（2）:256⁃273. doi: 10.1111/jdv.18720.
［16］	Boyd AS, Menter A. Erythrodermic psoriasis. Precipitating factors, course, and prognosis in 50 patients［J］. J Am Acad Dermatol, 1989,21（5 Pt 1）:985⁃991.
［17］	Creamer D, Walsh SA, Dziewulski P, et al. U.K. guidelines for the management of Stevens⁃Johnson syndrome/toxic epidermal necrolysis in adults 2016［J］. Br J Dermatol, 2016,174（6）:1194⁃1227. doi: 10.1111/bjd.14530.
［18］	Brüggen MC, Walsh S, Ameri MM, et al. Management of adult patients with drug reaction with eosinophilia and systemic symptoms: a delphi⁃based international consensus［J］. JAMA Dermatol, 2024,160（1）:37⁃44. doi: 10.1001/jamadermatol.2023. 4450.
［19］	Tetart F, Walsh S, Milpied B, et al. Acute generalized exanthematous pustulosis: European expert consensus for diagnosis and management［J］. J Eur Acad Dermatol Venereol, 2024,38（11）:2073⁃2081. doi: 10.1111/jdv.20232.
［20］	《脓疱型银屑病诊疗中国专家共识（2022版）》编写委员会专家组. 脓疱型银屑病诊疗中国专家共识（2022版）［J］. 中华皮肤科杂志, 2022,55（3）:187⁃195. doi: 10.35541/cjd.20210698.
［21］	Sassolas B, Haddad C, Mockenhaupt M, et al. ALDEN, an algorithm for assessment of drug causality in Stevens⁃Johnson Syndrome and toxic epidermal necrolysis: comparison with case⁃control analysis［J］. Clin Pharmacol Ther, 2010,88（1）:60⁃68. doi: 10.1038/clpt.2009.252.
［22］	中华医学会皮肤性病学分会药物不良反应研究中心. Stevens⁃Johnson综合征/中毒性表皮坏死松解症诊疗专家共识［J］. 中华皮肤科杂志, 2021,54（5）:376⁃381. doi: 10.35541/cjd.2020 1177.
［23］	Huang S, Anderson HJ, Lee JB. Paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome: PartⅡ. Diagnosis and management［J］. J Am Acad Dermatol, 2024,91（1）:13⁃22. doi: 10.1016/j.jaad.2023.08.084.
［24］	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治疗学组, 中国医疗保健国际交流促进会皮肤医学分会, 等. 中国天疱疮诊疗指南（2024版）［J］. 中华皮肤科杂志, 2024,57（10）:873⁃886. doi: 10.35541/cjd.20240222.
［25］	中国医师协会皮肤科医师分会, 中国中西医结合学会皮肤性病学分会. 红皮病型银屑病诊疗中国专家共识（2024版）［J］. 中华皮肤科杂志, 2024,57（5）:409⁃417. doi: 10.35541/cjd.2023 0570.
［26］	Bastuji⁃Garin S, Fouchard N, Bertocchi M, et al. SCORTEN: a severity⁃of⁃illness score for toxic epidermal necrolysis［J］. J Invest Dermatol, 2000,115（2）:149⁃153. doi: 10.1046/j.1523⁃1747.2000.00061.x.
［27］	Wang CW, Yang LY, Chen CB, et al. Randomized, controlled trial of TNF⁃α antagonist in CTL⁃mediated severe cutaneous adverse reactions［J］. J Clin Invest, 2018,128（3）:985⁃996. doi: 10.1172/JCI93349.
［28］	Olsson⁃Brown A, Yip V, Ogiji ED, et al. TNF⁃α⁃mediated keratinocyte expression and release of matrix metalloproteinase 9: putative mechanism of pathogenesis in Stevens⁃Johnson syndrome/toxic epidermal necrolysis［J］. J Invest Dermatol, 2023,143（6）:1023⁃1030.e7. doi: 10.1016/j.jid.2022.11.024.
［29］	Nordmann TM, Anderton H, Hasegawa A, et al. Spatial proteomics identifies JAKi as treatment for a lethal skin disease［J］. Nature, 2024,635（8040）:1001⁃1009. doi: 10.1038/s41586⁃024⁃08061⁃0.
［30］	Gibson A, Ram R, Gangula R, et al. Multiomic single⁃cell sequencing defines tissue⁃specific responses in Stevens⁃Johnson syndrome and toxic epidermal necrolysis［J］. Nat Commun, 2024,15（1）:8722. doi: 10.1038/s41467⁃024⁃52990⁃3.
［31］	Yang L, Shou YH, Li F, et al. Retrospective study of 213 cases of Stevens⁃Johnson syndrome and toxic epidermal necrolysis from China［J］. Burns, 2020,46（4）:959⁃969. doi: 10.1016/j.burns.2019.10.008.
［32］	Brauchle M, Fässler R, Werner S. Suppression of keratinocyte growth factor expression by glucocorticoids in vitro and during wound healing［J］. J Invest Dermatol, 1995,105（4）:579⁃584. doi: 10.1111/1523⁃1747.ep12323521.
［33］	Wei BM, Fox LP, Kaffenberger BH, et al. Drug⁃induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms. PartⅡ diagnosis and management［J］. J Am Acad Dermatol, 2024,90（5）:911⁃926. doi: 10.1016/j.jaad.2023. 02.073.
［34］	Kim D, Kobayashi T, Voisin B, et al. Targeted therapy guided by single⁃cell transcriptomic analysis in drug⁃induced hypersensitivity syndrome: a case report［J］. Nat Med, 2020,26（2）:236⁃243. doi: 10.1038/s41591⁃019⁃0733⁃7.
［35］	Parisi R, Shah H, Navarini AA, et al. Acute generalized exanthematous pustulosis: clinical features, differential diagnosis, and management［J］. Am J Clin Dermatol, 2023,24（4）:557⁃575. doi: 10.1007/s40257⁃023⁃00779⁃3.
［36］	Xuan Y, Jin S, Zhang C, et al. Rapid improvement in refractory acute generalized exanthematous pustulosis with spesolimab［J］. JAMA Dermatol, 2024,160（9）:1009⁃1012. doi: 10.1001/jamadermatol.2024.2311.
［37］	Rahbar Z, Daneshpazhooh M, Mirshams⁃Shahshahani M, et al. Pemphigus disease activity measurements: pemphigus disease area index, autoimmune bullous skin disorder intensity score, and pemphigus vulgaris activity score［J］. JAMA Dermatol, 2014,150（3）:266⁃272. doi: 10.1001/jamadermatol.2013.8175.
［38］	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.
［39］	Joly P, Horvath B, Patsatsi Α, et al. Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the European Academy of Dermatology and Venereology （EADV）［J］. J Eur Acad Dermatol Venereol, 2020,34（9）:1900⁃1913. doi: 10.1111/jdv.16752.
［40］	Lee MS, Yeh YC, Tu YK, et al. Network meta⁃analysis⁃based comparison of first⁃line steroid⁃sparing adjuvants in the treatment of pemphigus vulgaris and pemphigus foliaceus［J］. J Am Acad Dermatol, 2021,85（1）:176⁃186. doi: 10.1016/j.jaad. 2020.08.028.
［41］	Antiga E, Bech R, Maglie R, et al. S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology （EADV）［J］. J Eur Acad Dermatol Venereol, 2023,37（6）:1118⁃1134. doi: 10.1111/jdv.18931.
［42］	Anderson HJ, Huang S, Lee JB. Paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome: Part I. Clinical overview and pathophysiology［J］. J Am Acad Dermatol, 2024,91（1）:1⁃10. doi: 10.1016/j.jaad.2023.08.020.
［43］	Borradori L, Van Beek N, Feliciani C, et al. Updated S2 K guidelines for the management of bullous pemphigoid initiated by the European Academy of Dermatology and Venereology （EADV）［J］. J Eur Acad Dermatol Venereol, 2022,36（10）:1689⁃1704. doi: 10.1111/jdv.18220.
［44］	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.
［45］	Zhao L, Wang Q, Liang G, et al. Evaluation of dupilumab in patients with bullous pemphigoid［J］. JAMA Dermatol, 2023,159（9）:953⁃960. doi: 10.1001/jamadermatol.2023.2428.
［46］	Chebani R, Lombart F, Chaby G, et al. Omalizumab in the treatment of bullous pemphigoid resistant to first⁃line therapy: a French national multicentre retrospective study of 100 patients［J］. Br J Dermatol, 2024,190（2）:258⁃265. doi: 10.1093/bjd/ljad369.
［47］	Karakioulaki M, Eyerich K, Patsatsi A. Advancements in bullous pemphigoid treatment: a comprehensive pipeline update［J］. Am J Clin Dermatol, 2024,25（2）:195⁃212. doi: 10.1007/s40257⁃023⁃00832⁃1.
［48］	Chaudhary NS, Donnelly JP, Moore JX, et al. Association of baseline steroid use with long⁃term rates of infection and sepsis in the REGARDS cohort［J］. Crit Care, 2017,21（1）:185. doi: 10.1186/s13054⁃017⁃1767⁃1.
［49］	Rygård SL, Butler E, Granholm A, et al. Low⁃dose corticosteroids for adult patients with septic shock: a systematic review with meta⁃analysis and trial sequential analysis［J］. Intensive Care Med, 2018,44（7）:1003⁃1016. doi: 10.1007/s00134⁃018⁃5197⁃6.
［50］	Evans L, Rhodes A, Alhazzani W, et al. Surviving sepsis campaign: international guidelines for management of sepsis and septic shock 2021［J］. Intensive Care Med, 2021,47（11）:1181⁃1247. doi: 10.1007/s00134⁃021⁃06506⁃y.
［51］	Malpica L, Moll S. Practical approach to monitoring and prevention of infectious complications associated with systemic corticosteroids, antimetabolites, cyclosporine, and cyclophosphamide in nonmalignant hematologic diseases［J］. Hematology Am Soc Hematol Educ Program, 2020,2020（1）:319⁃327. doi: 10.1182/hematology.2020000116.
［52］	Rumancik B, Keele BJ, Rahnama⁃Moghadam S. Characterization of medical malpractice lawsuits relating to dermatologic emergencies in the inpatient and emergency settings［J］. J Am Acad Dermatol, 2022,86（2）:445⁃446. doi: 10.1016/j.jaad.2020. 02.015.
［53］	Zheng ZQ, Jiang C, Yu RL, et al. General characteristics, economic burden, causative drugs and medical errors associated with medical damage litigation involving severe cutaneous adverse drug reactions in China［J］. J Clin Pharm Ther, 2020,45（5）:1087⁃1097. doi: 10.1111/jcpt.13174.
［54］	Marsch A, Khodosh R, Porter M, et al. Implementing patient safety and quality improvement in dermatology. Part 1: patient safety science［J］. J Am Acad Dermatol, 2023,89（4）:641⁃654. doi: 10.1016/j.jaad.2022.01.049.
［55］	Jantararoungtong T, Tempark T, Koomdee N, et al. Genotyping HLA alleles to predict the development of Severe cutaneous adverse drug reactions （SCARs）: state⁃of⁃the⁃art［J］. Expert Opin Drug Metab Toxicol, 2021,17（9）:1049⁃1064. doi: 10.1080/17425255.2021.1946514.

[1]	Bao Shijie, Han Mei, Zhou Xiaoyong. Analysis of factors influencing the efficacy of etanercept in the treatment of toxic epidermal necrolysis based on literature review [J]. Chinese Journal of Dermatology, 2025, 58(4): 352-355.
[2]	Wang Sifan, He Chunxia, Jin Hongzhong. Clinical characteristics of and factors influencing Pneumocystis pneumonia secondary to drug-induced hypersensitivity syndrome [J]. Chinese Journal of Dermatology, 2025, 58(4): 315-321.
[3]	China Dermatologist Association, Combination of Traditional and Western Medicine Dermatology. Diagnosis and treatment of erythrodermic psoriasis: a Chinese expert consensus statement （2024） [J]. Chinese Journal of Dermatology, 2024, 57(5): 409-417.
[4]	Hu Xia, Liang Gaopeng, Wang Huan, Deng Sisi, Song Zhiqiang. Stevens-Johnson syndrome/toxic epidermal necrolysis: a retrospective study of 104 cases [J]. Chinese Journal of Dermatology, 2024, 57(12): 1114-1120.
[5]	Jiang Xiaoyan, Gao Min, Zhang Xiaoyan, Liu Ningyuan. Interleukin-17 and interleukin-23 inhibitors in the treatment of pustular psoriasis [J]. Chinese Journal of Dermatology, 2023, 56(7): 689-692.
[6]	Lian Jia, Chen Lixin, Wang Ying, Bi Tiantian, Song Yuantao, Li Qinfeng. Adalimumab combined with acitretin in the treatment of five children with generalized pustular psoriasis: a retrospective clinical observation [J]. Chinese Journal of Dermatology, 2022, 55(10): 916-918.
[7]	Long Hai, Jiang Li, Qiu Yueqi, Yao Nan, Liu Licong, Xie Yuming, Xiong Feng, Tan Siqi, Kuang Qiqi, You Ruixuan, Chai Ke, Luo Xin, Long Haojun, Xin Yue, Guo Ziyu, Wang Jiaqi, Tan Yixin, Zhang Qing, Zhang Guiying, Li Yaping, Su Yuwen, Xiao Rong, Lu Qianjin, . Clinical analysis of 1 057 patients with critical illnesses in a dermatological ward [J]. Chinese Journal of Dermatology, 2021, 54(9): 790-797.
[8]	Adverse Drug Reaction Research Center of Chinese Society of Dermatology. Expert consensus on the diagnosis and treatment of Stevens-Johnson syndrome/toxic epidermal necrolysis [J]. Chinese Journal of Dermatology, 2021, 54(5): 376-381.
[9]	Kang Li, Liu Yan, Zheng Yunyan, Ju Mei, Zhang Yamei, Bi Zhigang, Lu Guiqing. Clinical analysis of cutaneous delayed-type hypersensitivity caused by injection of equine tetanus antitoxin or equine anti-tetanus immunoglobulin F（ab′）₂ [J]. Chinese Journal of Dermatology, 2021, 54(3): 226-228.
[10]	Deng Sisi, Wang Huan, Yu Nanlan, Li Song, Song Zhiqiang. Clinical analysis of 45 cases of drug-induced hypersensitivity syndrome [J]. Chinese Journal of Dermatology, 2021, 54(2): 127-130.
[11]	Yao Cuiling, Wang Zihan, Hu Jingjing, Gao Yu, Ding Changling. Analysis of fever and drug-induced liver injury in 63 patients with severe drug eruptions [J]. Chinese Journal of Dermatology, 2021, 54(11): 984-989.
[12]	Xiao Yang, Zhang Xiaoguang. Lamotrigine-induced drug eruption [J]. Chinese Journal of Dermatology, 2021, 54(10): 927-930.
[13]	Gao Xuemei, Zhang Xingqi, Wang Fang. Application of enzyme?linked immunospot assay in the identification of culprit drugs in severe drug eruptions [J]. Chinese Journal of Dermatology, 2019, 52(6): 436-439.
[14]	Yu-Ping HUO. Acute generalized exanthematous pustulosis caused by lincomycin: a case report [J]. Chinese Journal of Dermatology, 2018, 51(7): 531-533.
[15]	. CD4+ and CD8+ T cell levels as well as clinical features in HIV-positive patients with drug eruption [J]. Chinese Journal of Dermatology, 2015, 48(12): 853-856.

Principles, current status, challenges and perspectives in the diagnosis and treatment of critical dermatological emergencies

PDF

Knowledge

Abstract

Cite this article

share this article

References ［55］

Related Articles 15

Metrics

Comments

Recommended 10