Corrigendum to ‘SJS/TEN 2019: From science to translation’ [J. Dermatol. Sci. 98/1 (2020) 2–12]

Chang, Wen Han; Abe, Riichiro; Anderson, Paul; Anderson, Wanpen; Ardern‐Jones, Michael R.; Beachkofsky, Thomas M.; Bellón, Teresa; Biala, Agnieszka; Bouchard, Charles S.; Cavalleri, Gianpiero L.; Chapman, Nicole M.; Chodosh, James; Choi, Hyon K.; Cibotti, Ricardo; Divito, Sherrie J.; Dewar, Karen M.; Dehaeck, Ulrike; Etminan, Mahyar; Forbes, Diane; Fuchs, Esther; Goldman, Jennifer L.; Holmes, James H.; Hope, Elyse A.; Hung, Shuen‐Iu; Hsieh, C C; Iovieno, Alfonso; Jagdeo, Julienne; Kim, Mee Kum; Koelle, David M.; Lacouture, Mario E.; Pallec, Sophie Le; Lehloenya, Rannakoe; Lim, Robyn; Lowe, Angie; McCawley, J.C.; McCawley, Julie; Micheletti, Robert G.; Mockenhaupt, Maja; Niemeyer, Katie; Norcross, Michael A.; Oboh, Douglas; Olteanu, Cristina; Pasieka, Helena B.; Peter, Jonathan; Pirmohamed, Munir; Rieder, Michael J.; Saeed, Hajirah N.; Shear, Neil H.; Shieh, Christine; Straus, Sabine M. J. M.; Sukasem, Chonlaphat; Sung, Cynthia; Trubiano, Jason A; Tsou, Sheng-Ying; Ueta, Mayumi; Volpi, Simona; Chen, Wan; Wang, Hongsheng; Wang, Zhaoqing; Weintraub, Jessica; Whale, Cindy; Wheatley, Lisa M.; Whyte-Croasdaile, Sonia; Williams, Kristina; Wright, Galen E.B.; Yeung, Sonia N.; Zhou, Li; Hung, Yi‐Teng; Phillips, Elizabeth; Carleton, Bruce

doi:10.1016/j.jdermsci.2021.09.008

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“…Die Äthiopathogenese der Erkrankungen ist nach wie vor nicht vollständig geklärt. Zugrunde liegt wahrscheinlich unter anderem ein gestörter Arzneimittelmetabolismus, welcher zu einer T-Zell-vermittelten Hypersensitivität der Haut führt [1,2,4]. So kommt es zu einer zellulären Immunreaktion.…”

Section: Diskussionunclassified

“…Eine groß angelegte kontrollierte Behandlungsstudie ist deswegen auch kaum umzusetzen [2]. Immunsuppressiva wie Kortikosteroide und Cyclosporin, Immunmodulatoren wie intravenöse Immunglobuline (IVIG) und Tumornekrosefaktoralpha-Antikörper wie Infliximab und Etanercept, Plasmapherese, Cyclophosphamid und der Granulocyte colony-stimulating Factor G-CSF werden als therapeutische Möglichkeiten angeführt [2,4,14] Literatur…”

Section: Diskussionunclassified

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Medikamenteninduziertes Stevens-Johnson-Syndrom und toxisch epidermale Nekrolyse als kutane Nebenwirkung in der Palliativversorgung

Wahl

Rolke

2023

Zeitschrift für Palliativmedizin

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ZusammenfassungDas Stevens-Johnson-Syndrom (SJS) und die toxisch epidermale Nekrolyse (TEN) gehören zu den schwerwiegendsten dermatologischen Krankheitsbildern. Sie sind immunvermittelte, kutane Nebenwirkungen, verlaufen oft hochakut und haben eine Mortalität von 30%. Die Inzidenz eines SJS/TEN liegt bei 1–2 Fällen/1000000, wobei das SJS bis zu 3-mal häufiger auftritt als ein TEN. Als Hochrisiko-Medikamente für das Auftreten eines SJS/TEN wurden unter anderem Sulfonamidantibiotika, Antiepileptika, nichtsteroidale Antiphlogistika vom Oxicam-Typ und Allopurinol identifiziert. In der Literatur werden verschiedenste weitere Substanzen benannt, die diese Hautreaktion verursachen können. Es ist einer der wichtigsten Behandlungsschritte bei einem SJS/TEN, das möglicherweise auslösende Medikament zu identifizieren und abzusetzen. Bei Polypharmazie – insbesondere auch bei Palliativpatientinnen und -patienten – kann das eine Herausforderung sein. Die systemische Behandlung eines SJS/TEN wird in der Fachwelt kontrovers diskutiert. In jedem Fall wichtig ist eine adäquate Lokal- und additive Therapie. Die Behandlung eines SJS/TEN sollte multidisziplinär erfolgen. Wir berichten über den Fall einer 77-jährigen Palliativpatientin, die innerhalb von 2 Wochen eine toxisch epidermale Nekrolyse entwickelte. Als möglicherweise auslösende Medikamente konnten wir Hydrochlorothiazid, Sertralin und Metamizol identifizieren.

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Section: Diskussionunclassified

Medikamenteninduziertes Stevens-Johnson-Syndrom und toxisch epidermale Nekrolyse als kutane Nebenwirkung in der Palliativversorgung

Wahl

Rolke

2023

Zeitschrift für Palliativmedizin

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Spectrum of offending drugs and cutaneous adverse drug reactions requiring hospitalisation in a tertiary South African hospital in TB/HIV endemic setting

Konyana,

Teixeira,

Pirjol

et al. 2024

Front. Allergy

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IntroductionCutaneous immune-mediated adverse drug reactions are more prevalent in people with human immunodeficiency virus (PWH). Severe cutaneous adverse drug reactions (SCAR) are a life-threatening subset of cutaneous adverse drug reactions (CADRs) and a significant public health issue in settings endemic for human immunodeficiency virus and tuberculosis. However, limited data are available on CADR requiring hospitalisation in African settings. The aim of this study is to describe the epidemiology, offending drugs and outcomes of CADRs requiring admission to a South African tertiary dermatology service.MethodsRetrospective folder review was conducted on all CADRs requiring hospitalisation at Nelson Mandela Academic Hospital in Mthatha, Eastern Cape, South Africa between 30 July 2015 and 15 December 2022. This data was compared to prospective inclusion of CADR admissions between 03 March 2021 and 09 April 2024 as part of the Immune-Mediated Adverse Drug Reactions (IMARI) Registry and Biorepository and AFRISCAR consortium. Where possible, phenotype and drug causality assessment was performed through RegiSCAR, or Naranjo and/or ALDEN scoring respectively.ResultsCADR admissions included 122 cases: 89 and 33 in the retrospective and prospective cohorts respectively. The commonest SCAR phenotype was Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) at 59.8% (73/122), although other validated SCAR phenotypes included drug reaction with eosinophilia and systemic symptoms (DRESS), acute generalized exanthematous pustulosis (AGEP) and generalized fixed bullous drug eruption (GBFDE). Cutaneous presentations included typical and atypical SCAR features against a background Fitzpatrick skin tones of type IV and above. Amongst the retrospective cohort 16.9% (15/89) of phenotypes were unclassifiable due to lack of photographs. The overall median (IQR) age was 38 (25–50) years, 50.8% (62/122) were male and 60.7% (74/122) were PWH [median (IQR) CD4T-cell count of 267 (76–470) cells/mm3]. The commonest offending drugs included cotrimoxazole in 24.6% (30/122); and anti-retroviral therapy (ART) in 13.9% (17/122). No offending drug could be identified in 24.7% (22/89) of the retrospective cohort. The median (IQR) length of hospital stay for validated SCAR was 13 (8–21) days for the retrospective cohort and 19 (13–28) days for the prospective cohort (p = 0.03). The median (IQR) length of hospital stay for non-SCAR was 9 (5–13) days for the retrospective cohort and 11 (9–16) days for the prospective cohort.ConclusionTypical and atypical presentations of SCAR were represented in this vulnerable South African cohort of predominantly PWH. SJS/TEN was the commonest phenotype, and cotrimoxazole the most frequent offending drug. This data emphasises the need for prospective data collection across a diverse African population for valid SCAR phenotyping and drug causality assessment.

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Stevens Johnson Syndrome: Past, Present, and Future Directions Gynecologic Manifestations and Management in SJS/TEN

2022

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Stevens Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are severe mucocutaneous hypersensitivity disorders characterized by sudden onset epidermal necrosis. Acute manifestations of SJS/TEN often include vulvovaginal erosions, ulcerations, vaginal discharge, bleeding, vaginal pain, dysuria, and urinary retention. If not treated, this can lead to complications such as vulvovaginal adhesions, vaginal stenosis or dryness, pain, dyspareunia, bleeding, and adenosis. Even with adequate treatment, there are lasting impacts including difficulty with vaginal exams and psychological distress. Early recognition and treatment of vulvovaginal involvement are crucial to preventing severe sequelae. Despite the potentially devastating consequences of genitourinary involvement of SJS/TEN, involvement of the mucocutaneous surfaces of the vulva and vagina is inconsistently documented, and protocols for treatment and follow-up are not well-established. The treatment of vulvovaginal involvement relies largely on expert opinion, and there is little data on the efficacy of suggested management. The goal of this review was to identify whether establishing a clinical pathway increased treatment of vulvovaginal SJS/TEN and to optimize our standardized protocol to prevent genitourinary sequelae.MethodsWe conducted a retrospective chart review of female patients with SJS/TEN at Harborview Medical Center, University of Washington from 2008 to 2021. Demographic and clinical data including gynecologic consultation, exam findings, treatment regimens, and outpatient follow-up were collected from the electronic medical record. We compared data before and after implementation of a clinical care pathway in 2017.ResultsWe reviewed a total of 88 charts of women with possible SJS/TEN between 2008 and 2021. Of these 88 charts, 77 were found to have clear biopsy proven diagnosis of SJS/TEN. A total of 42 patients were found to have vulvovaginal involvement (55%) and gynecology was consulted in 43% of cases. 50% of patients (n = 21) with vulvovaginal involvement were recommended treatment with vaginal dilators and steroid ointment and 34% of patients with genital involvement received no treatment.Between 2008 and May of 2017 (pre-protocol), we found 55 patients with SJS/TEN. 55% of patients (n = 29) had vulvovaginal involvement (n = 26 vulvar, n = 21 vaginal). Gynecology was only consulted in 26% (n = 14) of patients. Of the 21 females with vaginal involvement, only 38% (n = 8) had dilators/vaginal molds with steroid ointment recommended. Of the 26 females with vulvar involvement, 31% (n = 8) had no vulvar treatment recommendations with the remaining 69% having some documentation that ranged from gauze placement only (19%) to topical lidocaine, barrier cream, antibiotic or antifungal cream/ ointment, lubricant, or topical steroid ointment (50%). Menstrual suppression was recommended in 38% (n = 9) of menstruating females. An antifungal medication was only prescribed in 4% of patients.Following implementation of the clinical pathway for the treatment of SJS/TEN in 2017, 22 females with SJS/TEN were identified. 72% (n = 16) had documented vulvovaginal involvement (n = 16 vulvar, n = 9 vaginal). Gynecology consultations took place in 86% (n = 19) of patients. We identified several improvements after implementation of the protocol. Gynecology consults overall increased from 26% pre-, to 86% post-protocol. For patients with vulvovaginal involvement, consultations were completed in 93% compared to 50% prior to protocol. Of note, the finding of vulvovaginal lesions increased from 53 to 72%. Dilator use with topical steroid ointment was consistently recommended, as was antifungal use and menstrual suppression.ConclusionHaving a protocol in place for treatment of female patients with SJS/TEN increased the consistency of Gynecologic consultation and the documentation and treatment of vulvovaginal SJS/TEN. We identified the need to improve clinical follow-up after discharge from the hospital, which could be arranged as multidisciplinary visits and would be a good option to assess long-term outcomes (pain, sexual activity, etc.). With regards to future directions, we are in the process of assessing long-term data on quality of life and sexual functioning. The impact of treatment in the acute setting on the development of chronic sequelae needs to be established, as does the management of long-term sequelae like vaginal dryness, pain, dyspareunia. The role of local estrogen and vaginal laser still needs to be explored. Pelvic floor physical therapy might play a significant role in rehabilitation and has yet to be studied.

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Corrigendum to ‘SJS/TEN 2019: From science to translation’ [J. Dermatol. Sci. 98/1 (2020) 2–12]

Cited by 3 publications

References 0 publications

Medikamenteninduziertes Stevens-Johnson-Syndrom und toxisch epidermale Nekrolyse als kutane Nebenwirkung in der Palliativversorgung

Medikamenteninduziertes Stevens-Johnson-Syndrom und toxisch epidermale Nekrolyse als kutane Nebenwirkung in der Palliativversorgung

Spectrum of offending drugs and cutaneous adverse drug reactions requiring hospitalisation in a tertiary South African hospital in TB/HIV endemic setting

Stevens Johnson Syndrome: Past, Present, and Future Directions Gynecologic Manifestations and Management in SJS/TEN

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