Clinical and anamnestic analysis of patients with Stevens–Johnson syndrome/toxic epidermal necrolysis hospitalised in Moscow. Development of a prognostic model of unfavourable outcomes

Ekaterina A. Nikitina; Никитина Екатерина Андреевна; Alexander D. Dushkin; Душкин Александр Дмитриевич; Yuriy V. Streltsov; Стрельцов Юрий Владимирович; Sergey S. Andreev; Андреев Сергей Сергеевич; Tatyana S. Kruglova; Круглова Татьяна Сергеевна; Ulyana A. Markina; Маркина Ульяна Алексеевна; Marina S. Lebedkina; Лебедкина Марина Сергеевна; Maryana A. Lysenko; Лысенко Марьяна Анатольевна; Darya S. Fomina; Фомина Дарья Сергеевна

doi:10.36691/RJA16995

Clinical and anamnestic analysis of patients with Stevens–Johnson syndrome/toxic epidermal necrolysis hospitalised in Moscow. Development of a prognostic model of unfavourable outcomes

Authors: Nikitina E.A.¹^,2, Dushkin A.D.¹^,3^,4, Streltsov Y.V.¹, Andreev S.S.¹, Kruglova T.S.¹, Markina U.A.¹, Lebedkina M.S.¹, Lysenko M.A.¹^,5, Fomina D.S.¹^,2^,6
Affiliations:
1. Moscow City Hospital 52
2. The First Sechenov Moscow State Medical University (Sechenov University)
3. National Medical Research Center for High Medical Technologies — Central Military Clinical Hospital named after A.A. Vishnevsky
4. S.M. Kirov Military Medical Academy
5. The Russian National Research Medical University named after N.I. Pirogov
6. Astana Medical University
Pages: 233-247
Section: Original studies
Submitted: 27.01.2025
Accepted: 20.06.2025
Published: 02.08.2025
URL: https://rusalljournal.ru/raj/article/view/16995
DOI: https://doi.org/10.36691/RJA16995
ID: 16995

Cite item

Full Text

Open Access
Restricted Access

Access granted
Restricted Access

Subscription or Fee Access

Abstract
Full Text
About the authors
References
Supplementary files
Statistics

Abstract

BACKGROUND: Stevens–Johnson syndrome and toxic epidermal necrolysis are severe life-threatening conditions characterized by massive lesions of the skin and mucosa. At present, considering the high mortality rate, one of the most promising areas of research is the study of predictors of the severity of the pathology, since prognosis of the disease can further influence the choice of treatment strategy.

AIM: Determination of epidemiological features, identification of clinical and laboratory predictors of the disease severity, and construction of a prognostic model for patients with Stevens–Johnson syndrome and toxic epidermal necrolysis within the framework of analysis of electronic medical records of Moscow.

MATERIALS AND METHODS: The study was based on a retrospective analysis of medical records of patients with SJS/TEN from 2020 to 2023. Initially, 230 individuals over 18 years of age were included in the analysis. As a result of selection from the primary cohort, 122 patients satisfying the criteria for the diagnosis of Stevens–Johnson syndrome and toxic epidermal necrolysis were included in the final analysis. Patients did not undergo additional follow-up as part of this study.

RESULTS: In the analyzed cohort, a prevalence of female patients (n = 72; 59.01 %) over male patients (n = 50; 40.99 %) was observed. Lethal outcome was recorded in 27 (22.13 %) patients, of which 21 (77.8 %) had a verified diagnosis of toxic epidermal necrolysis, which was associated with a higher incidence of death compared to Stevens–Johnson syndrome (n = 6 (p = 0.001)). It was found that 112 (91.8 %) cases were likely associated with medication use, while 10 (8.2 %) presented a verified infectious agent Mycoplasma pneumoniae. Antiepileptic drugs were the most frequent cause of drug-induced Stevens–Johnson syndrome/toxic epidermal necrolysis (n = 62; 55.4 %). Based on the analysed clinical and laboratory data, a prognostic model was developed to determine the probability of lethal outcome, including decreased serum bicarbonate, increased levels of c-reactive protein, fibrinogen, fever, hypoalbuminemia.

CONCLUSION: Stevens–Johnson syndrome and toxic epidermal necrolysis are rare conditions with a high mortality rate and high risk of disabling complications. Early verification of the diagnosis and stratification of patients by severity group is optimal for the choice of treatment tactics; however, further work is currently required to standardise the assessment of the severity of patients with Stevens-Johnson syndrome and toxic epidermal necrolysis.

Keywords

Stevens–Johnson syndrome, toxic epidermal necrolysis, predictive modelling, anticonvulsant

Full Text

MD, Dr. Sci. (Medicine), Professor

Россия, Moscow; Moscow

Darya S. Fomina

Moscow City Hospital 52; The First Sechenov Moscow State Medical University (Sechenov University); Astana Medical University

Email: daria_fomina@mail.ru
ORCID iD: 0000-0002-5083-6637
SPIN-code: 3023-4538

MD, Cand. Sci. (Medicine), Assistant Professor

Россия, Moscow; Moscow; Republic of Kazakhstan, Astana

References

Fukasawa T, Takahashi H, Kameyama N, et al. Development of an electronic medical record-based algorithm to identify patients with Stevens–Johnson syndrome and toxic epidermal necrolysis in Japan. PLoS One. 2019;14(8):e0221130. doi: 10.1371/journal.pone.0221130
Duong TA, Valeyrie-Allanore L, Wolkenstein P, Chosidow O. Severe cutaneous adverse reactions to drugs. Lancet. 2017;390(10106):1996–2011. doi: 10.1016/S0140-6736(16)30378-6 EDN: YDEHZJ Erratum in: Lancet. 2017;390(10106):1948. doi: 10.1016/S0140-6736(17)31656-2
Frey N, Jossi J, Bodmer M, et al. The epidemiology of Stevens–Johnson syndrome and toxic epidermal necrolysis in the UK. J Invest Dermatol. 2017;137(6):1240–1247. doi: 10.1016/j.jid.2017.01.031
Hsu DY, Brieva J, Silverberg NB, Silverberg JI. Morbidity and mortality of Stevens–Johnson syndrome and toxic epidermal necrolysis in United States adults. J Invest Dermatol. 2016;136(7):1387–1397. doi: 10.1016/j.jid.2016.03.023
Paulmann M, Mockenhaupt M. Severe drug-induced skin reactions: clinical features, diagnosis, etiology, and therapy. J Dtsch Dermatol Ges. 2015;13(7):625–645. doi: 10.1111/ddg.12747
Gueudry J, Roujeau JC, Binaghi M, et al. Risk factors for the development of ocular complications of Stevens–Johnson syndrome and toxic epidermal necrolysis. Arch Dermatol. 2009;145(2):157–162. doi: 10.1001/archdermatol.2009.540
Crowder CA, Jeney SES, Kraus CN, et al. Vulvovaginal involvement in Stevens–Johnson syndrome and toxic epidermal necrolysis: management and techniques used to reduce gynecologic sequelae. Int J Dermatol. 2022;61(2):158–163. doi: 10.1111/ijd.15676 EDN: NOHFGB
Bastuji-Garin S, Fouchard N, Bertocchi M, et al. SCORTEN: a severity-of-illness score for toxic epidermal necrolysis. J Invest Dermatol. 2000;115(2):149–153. doi: 10.1046/j.1523-1747.2000.00061.x
Hu CH, Chang NJ, Liu EK, et al. SCORTEN and impaired renal function related to mortality of toxic epidermal necrolysis syndrome patients in the Asian population. J Eur Acad Dermatol Venereol. 2013;27(5):628–633. doi: 10.1111/j.1468-3083.2012.04502.x
Micheletti RG, Chiesa-Fuxench Z, Noe MH, et al. Stevens–Johnson syndrome/toxic epidermal necrolysis: a multicenter retrospective study of 377 adult patients from the United States. J Invest Dermatol. 2018;138(11):2315–2321. doi: 10.1016/j.jid.2018.04.027 Erratum in: J Invest Dermatol. 2019;139(2):495–496. doi: 10.1016/j.jid.2018.11.013
Sekula P, Liss Y, Davidovici B, et al. Evaluation of SCORTEN on a cohort of patients with Stevens–Johnson syndrome and toxic epidermal necrolysis included in the RegiSCAR study. J Burn Care Res. 2011;32(2):237–245. doi: 10.1097/BCR.0b013e31820aafbc
Noe MH, Rosenbach M, Hubbard RA, et al. Development and validation of a risk prediction model for in-hospital mortality among patients with Stevens–Johnson syndrome/toxic epidermal necrolysis-ABCD-10. JAMA Dermatol. 2019;155(4): 448–454. doi: 10.1001/jamadermatol.2018.5605
Torres-Navarro I, Briz-Redón Á, Botella-Casas G, et al. Accuracy of SCORTEN and ABCD-10 to predict mortality and the influence of renal function in Stevens–Johnson syndrome/toxic epidermal necrolysis. J Dermatol. 2020;47(10):1182–1186. doi: 10.1111/1346-8138.15490 EDN: VWARHN
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. Clin Pharmacol Ther. 2010;88(1):60–68. doi: 10.1038/clpt.2009.252
Yamane Y, Matsukura S, Watanabe Y, et al. Retrospective analysis of Stevens–Johnson syndrome and toxic epidermal necrolysis in 87 Japanese patients – treatment and outcome. Allergol Int. 2016;65(1):74–81. doi: 10.1016/j.alit.2015.09.001
Wasuwanich P, So JM, Chakrala TS, et al. Epidemiology of Stevens–Johnson syndrome and toxic epidermal necrolysis in the United States and factors predictive of outcome. JAAD Int. 2023;13:17–25. doi: 10.1016/j.jdin.2023.06.014 EDN: GIKANS
Cheung CMT, Chang MM, Li JJX, Chan AWS. Stevens–Johnson syndrome and toxic epidermal necrolysis in Hong Kong. Hong Kong Med J. 2024;30(2):102–109. doi: 10.12809/hkmj2210131
Revuz J, Penso D, Roujeau JC, et al. Toxic epidermal necrolysis. Clinical findings and prognosis factors in 87 patients. Arch Dermatol. 1987;123(9):1160–1165. doi: 10.1001/archderm.123.9.1160
White KD, Abe R, Ardern-Jones M, et al. SJS/TEN 2017: building multidisciplinary networks to drive science and translation. J Allergy Clin Immunol Pract. 2018;6(1):38–69. doi: 10.1016/j.jaip.2017.11.023 EDN: YFCDNB
Marks ME, Botta RK, Abe R, et al. Updates in SJS/TEN: collaboration, innovation, and community. Front Med. 2023;10:1213889. doi: 10.3389/fmed.2023.1213889 EDN: KJGZMX
Yamane Y, Aihara M, Ikezawa Z. Analysis of Stevens–Johnson syndrome and toxic epidermal necrolysis in Japan from 2000 to 2006. Allergol Int. 2007;56(4):419–425. doi: 10.2332/allergolint.O-07-483
Charlton OA, Harris V, Phan K, et al. Toxic epidermal necrolysis and Steven–Johnson syndrome: a comprehensive review. Adv Wound Care (New Rochelle). 2020;9(7):426–439. doi: 10.1089/wound.2019.0977 EDN: RGWKZX
Mani R, Monteleone C, Schalock PC, et al. Rashes and other hypersensitivity reactions associated with antiepileptic drugs: a review of current literature. Seizure. 2019;71:270–278. doi: 10.1016/j.seizure.2019.07.01 EDN: DPEUQP
Sibbald C, Putterman E, Micheletti R, et al. Retrospective review of drug-induced Stevens–Johnson syndrome and toxic epidermal necrolysis cases at a pediatric tertiary care institution. Pediatr Dermatol. 2020;37(3):461–466. doi: 10.1111/pde.14118 EDN: ISYIYF
Guégan S, Bastuji-Garin S, Poszepczynska-Guigné E, et al. Performance of the SCORTEN during the first five days of hospitalization to predict the prognosis of epidermal necrolysis. J Invest Dermatol. 2006;126(2):272–276. doi: 10.1038/sj.jid.5700068
Dobry AS, Himed S, Waters M, Kaffenberger BH. Scoring assessments in Stevens–Johnson syndrome and toxic epidermal necrolysis. Front Med. 2022;9:883121. doi: 10.3389/fmed.2022.883121 EDN: DJYLYV
Nizamoglu M, Ward JA, Frew Q, et al. Improving mortality outcomes of Stevens Johnson syndrome/toxic epidermal necrolysis: a regional burns centre experience. Burns. 2018;44(3):603–611. doi: 10.1016/j.burns.2017.09.015
Hasegawa A, Abe R. Recent advances in managing and understanding Stevens–Johnson syndrome and toxic epidermal necrolysis. F1000Res. 2020;9:F1000 Faculty Rev–612. doi: 10.12688/f1000research.24748.1 EDN: KKOUAN