Dear Editor, Stevens–Johnson syndrome (SJS)/Toxic Epidermal Necrolysis (TEN) is a rare but life-threatening illness affecting 1–6 people per million per year in the United States (US). With best supportive care, approximately 80–85% survive acute hospitalization. However, due to inconsistent long-term follow-up for SJS/TEN, there is a lack of detailed information on long-term mental and physical health.
The SJS Survivors Study (Vanderbilt University Medical Center IRB#19135) identified US survivors using SJS Foundation outreach, physician referrals, and social media postings.1 After obtaining written consent, enrolled participants meeting inclusion criteria underwent secondary screening. Participant’s medical records were obtained, with dermatologist adjudication to confirm SJS/TEN diagnosis and probable drug causality. Participants with confirmed SJS/TEN completed online questionnaires related to their survivorship experience including RegiSCAR 5-year questionnaire, PROMIS 29, Major Life Changing Decisions Profile, EQ5D-5L, Impact of Events Scale, Dermatology Life Quality Index (DLQI), and Skindex-29.
Medians with interquartile ranges (IQR) were used to summarize demographics and questionnaire responses. These were stratified by years since their reported SJS/TEN diagnosis verified by their medical record with all data collected between September 2019 and August 2024. Analyses were done using R version 4.4.2 (2022-10-31).
Of the 385 enrolled participants with self-reported SJS/TEN, 112 were confirmed cases of SJS/TEN by dermatologist adjudication and included in the study. Respondents with confirmed SJS/TEN were predominantly female (90/112, 80%) and white (94/112, 84%). The median age at the time of the survey was 36 (27.1,52.6) with a median age of SJS/TEN diagnosis of 27 (16, 41.8). Most respondents had medical record documented body surface area detachment ≥10% (88/112, 79%).
Participants reported substantial long-term morbidity, disability and quality of life effects of SJS/TEN. 31 of 112 (28%) participants reported moderate skin complaints. 50 of 112 (45%) participants reported inability to work because of their SJS/TEN, including 18 of 39 (46%) whose SJS/TEN reaction was more than 10 years prior to the survey (Table 1, Figshare). Most participants report ongoing fear of medication (85/112, 76%), medication avoidance (84/112, 75%) and lasting life-changing changing effects (68/112, 61%) including careers (18/112, 16%).
Table 1.
Characteristics of survivors of Stevens–Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) stratified by years since reaction
| Characteristic | Overall (n = 112) | ≤ 5 years since SJS/TEN (n = 52) | 5–10 years since SJS/TEN (n = 21) | > 10 years since SJS/TEN (n = 39) |
|---|---|---|---|---|
| Age at survey (years), median (IQR) | 36 (27–53) | 33 (24–45) | 46 (30–59) | 37 (32–58) |
| Age at SJS/TEN (years), median (IQR) | 27 (16–42) | 29 (21–42) | 38 (24–49) | 16 (9.5–31) |
| Race (n) | ||||
| Asian | 9 (8) | 8 (15) | 0 (0) | 1 (3) |
| Black | 8 (7) | 4 (8) | 1 (5) | 3 (8) |
| Middle Eastern | 1 (1) | 0 (0) | 1 (5) | 0 (0) |
| White | 94 (84) | 40 (77) | 19 (90) | 35 (90) |
| Hispanic/Latino (self-report) (n) | 8 (7) | 7 (13) | 1 (5) | 0 (0) |
| SJS < 10% BSA | 24 (21) | 13 (25) | 3 (14) | 8 (21) |
| SJS/TEN ≥ 10% BSA | 88 (79) | 39 (75) | 18 (86) | 31 (79) |
| Effect on life | ||||
| None | 33 (29) | 14 (27) | 8 (38) | 11 (28) |
| Moderate | 18 (16) | 5 (10) | 3 (14) | 10 (26) |
| Extreme | 1 (1) | 1 (2) | 0 (0) | 0 (0) |
| Unable to work | 50 (45) | 23 (44) | 9 (43) | 18 (46) |
| Impaired sexuality | 37 (33) | 16 (31) | 5 (24) | 16 (41) |
| Medication avoidance | 84 (75) | 38 (73) | 18 (86) | 28 (72) |
| DLQI | 2.0 (1.0–5.2) | 3.0 (1.0–6.0) | 2.0 (1.0–9.0) | 2.0 (0.5–3.5) |
| Skindex-29 domain | ||||
| Emotions | 20 (5–42) | 30 (10–50) | 16 (5–41) | 12 (0–28) |
| Symptoms | 21.4 (7.1–39.3) | 25.0 (9.8–40.2) | 30.4 (10.7–47.3) | 17.9 (7.1–35.7) |
| Functioning | 6.2 (0.0–22.9) | 9.4 (0.0–27.6) | 5.2 (1.6–37.5) | 2.1 (0.0–16.7) |
| Median IQR | ||||
| EQ-5D-5L, median (IQR) | 0.81 (0.61–0.89) | 0.80 (0.64–0.88) | 0.88 (0.71–0.94) | 0.81 (0.57–0.88) |
Data are presented as n (%) unless otherwise stated. BSA, body surface area; DLQI, Dermatology Life Quality Index; IQR, interquartile range.
Interestingly, lasting consequences of SJS/TEN appeared to be unrelated to ongoing skin-specific morbidity or quality of life effects: median DLQI and Skindex-29 scores were each only two, representing minimal effects on skin-specific health-related quality of life. For major life changing decisions profile, the scores on individual sections and overall reflect that SJS/TEN is a life-changing event and that the effect persists over time, particularly impacting career, physical, and social health.
This is one of the largest long-term studies following an SJS/TEN reaction that reports long-term survey-based sequelae and adds to the understanding of SJS/TEN as a condition with chronic complications beyond the acute reaction. A single-center study from Canada surveyed 44 SJS/TEN survivors and found similar effects on employment; interestingly they found more apparent lasting effects on skin-specific quality of life measured using the DLQI and Skindex-29.2 That study also reported a wide range of long-term physical complications after SJS/TEN.3 A US survey study (n=121) of adults who survived SJS/TEN at one of 11 academic hospitals found high rates of post-traumatic stress disorder.4 They also found high rates of fear of medications, but in contrast with our findings, only 30% of their sample population actually avoided prescription medications. In 2023, a multidisciplinary consensus exercise recommended several post-discharge care strategies for SJS/TEN survivors as a step towards improving these outcomes.5
Our studies strengths included outreach through social media and patient support groups to identify US SJS/TEN survivors irrespective of the geography, the medical center where they were cared for, and their health insurance status. SJS/TEN diagnosis was dermatologist-adjudicated, adding validity to each participant’s reaction. Limitations of our study include the largely female and White respondents, and risks of self-selection bias. Further, our method of patient selection has the potential for bias towards patients who have had long term sequele or QoL inpact. Therefore, care needs to be given before comparing these results to other patient recruitment methods. Additionally, our predominantly online recruitment may have biased our sample towards participants who are more engaged and identified as having chronic SJS/TEN, and therefore whose disease has more of an impact on their lives.
We report significant long-term life impacts of SJS/TEN among survivors in the US. As medical care continues to improve survival after SJS/TEN, measures to improve and understand optimal follow-up and long-term care after acute SJS/TEN reactions are needed.
Funding sources:
National Institutes of Health, RHG010863-01; Dr. Phillips reports grants from National Institutes of Health (R01HG010863, R01AI152183, U01AI154659, R13AR082704) and from the National Health and Medical Research Council of Australia; Dr. Drucker has received research grants to his institution from the National Eczema Association, Eczema Society of Canada, Canadian Dermatology Foundation, Canadian Institutes for Health Research, US National Institutes of Health and Physicians Services Incorporated Foundation; Dr. Kaffenberger has received research funding from Biogen, Merck, Boehringer-Ingelheim, BMS, Onquality, InflaRx, The National Psoriasis Foundation, and Dermatology Foundation; Dr. Martin-Pozo reports a grant from the National Institutes of Health (R13AR082704).
Footnotes
Conflicts of interest: Dr. Phillips receives royalties and consulting fees from UpToDate and Uptodate Lexidrug (where she is a Drug Allergy Section Editor and section author) and has received consulting fees from Janssen, Vertex, Verve, Servier, Rapt and Esperion. E.J.P. is co-director of IIID Pty Ltd, which holds a patent for HLA-B*57:01 testing for abacavir hypersensitivity, and E.J.P has a patent pending for detection of HLA-A*32:01 in connection with diagnosing drug reaction with eosinophilia and systemic symptoms to vancomycin. For these patents she does not receive any financial remuneration, and neither are related to the submitted work. Dr. Drucker has received compensation from the British Journal of Dermatology (Deputy Editor), American Academy of Dermatology (guidelines writer), Canadian Dermatology Today (manuscript writer), and National Eczema Association (consultant) Canada’s Drug Agency (consultant). Dr. Kaffenberger has received speaking fees from Boehringer-Ingelheim, and consulting fees from Biogen, Elsevier, Eli Lilly and Company, Novartis, Novocure, and ADC therapeutics.
Ethics statement: This study was approved by the Vanderbilt University Medical Center Institutional Review Board, with IRB number #19135.
Patient consent: Written patient consent for publication was obtained.
Data availability:
The data underlying this article will be shared on reasonable request to the corresponding author.
References
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Data Availability Statement
The data underlying this article will be shared on reasonable request to the corresponding author.