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Kridin, Khalaf; Bruggen, Marie-Charlotte; Chua, Ser-Ling; Bygum, Anette; Walsh, Sarah; Nageli, Mirjam C.; Kucinskiene, Vesta; French, Lars; Tetart, Florence; Didona, Biagio; Milpied, Brigitte; Ranki, Annamari; Salavastru, Carmen; Brezinova, Eva; Divani-Patel, Sapna; Lorentzen, Tine; Nagel, Julie Loft; Valiukeviciene, Skaidra; Karpaviciute, Viktorija; Tiplica, George-Sorin; Oppel, Eva; Oschmann, Anna; de Prost, Nicolas; Vorobyev, Artem and Ingen-Housz-Oro, Saskia (2021): Assessment of Treatment Approaches and Outcomes in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Insights From a Pan-European Multicenter Study. In: Jama Dermatology, Vol. 157, No. 10: pp. 1182-1190

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IMPORTANCE Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe drug reactions associated with a high rate of mortality and morbidity. There is no consensus on the treatment strategy. OBJECTIVE To explore treatment approaches across Europe and outcomes associated with the SJS/TEN disease course, as well as risk factors and culprit drugs. DESIGN, SETTING, AND PARTICIPANTS A retrospective pan-European multicenter cohort study including 13 referral centers belonging to the ToxiTEN ERN-skin subgroup was conducted. A total of 212 adults with SJS/TEN were included between January 1, 2015, and December 31, 2019, and data were collected from a follow-up period of 6 weeks. MAIN OUTCOMES AND MEASURES Risk factors for severe acute-phase complications (acute kidney failure, septicemia, and need for mechanical ventilation) and mortality 6 weeks following admission were evaluated using a multivariable-adjusted logistic regression model. One tool used in evaluation of severity was the Score of Toxic Epidermal Necrolysis (SCORTEN), which ranges from 0 to 7, with 7 the highest level of severity. RESULTS Of 212 patients (134 of 211 [63.7%] women;mean [SD] age, 51.0 [19.3] years), the mean (SD) body surface area detachment was 27% (32.8%). In 176 (83.0%) patients, a culprit drug was identified. Antibiotics (21.2%), followed by anticonvulsants (18.9%), nonsteroidal anti-inflammatory drugs (11.8%), allopurinol (11.3%), and sulfonamides (10.4%), were the most common suspected agents. Treatment approaches ranged from best supportive care only (38.2%) to systemic glucocorticoids (35.4%), intravenous immunoglobulins (23.6%), cyclosporine (10.4%), and antitumor necrosis factor agents (3.3%). Most patients (63.7%) developed severe acute-phase complications. The 6-week mortality rate was 20.8%. Maximal body surface area detachment (>= 30%) was found to be independently associated with severe acute-phase complications (fully adjusted odds ratio [OR], 2.49;95% CI, 1.21-5.12;P = .01) and SCORTEN greater than or equal to 2 was significantly associated with mortality (fully adjusted OR, 10.30;95% CI, 3.82-27.78;P < .001). Cyclosporine was associated with a higher frequency of greater than or equal to 20% increase in body surface area detachment in the acute phase (adjusted OR, 3.44;95% CI, 1.12-10.52;P = .03) and an increased risk of infections (adjusted OR, 7.16;95% CI, 1.52-33.74;P = .01). Systemic glucocorticoids and intravenous immunoglobulins were associated with a decreased risk of infections (adjusted OR, 0.40;95% CI, 0.18-0.88;P = .02). No significant difference in 6-week mortality was found between treatment groups. CONCLUSIONS AND RELEVANCE This cohort study noted differences in treatment strategies for SJS/TEN in Europe;the findings suggest the need for prospective therapeutic studies to be conducted and registries to be developed.

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