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Diagnosis and Management of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Pediatric Patients: A Systematic Review of Clinical Guidelines and Consensus Statements.

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Τίτλος: Diagnosis and Management of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Pediatric Patients: A Systematic Review of Clinical Guidelines and Consensus Statements.
Συγγραφείς: Creighton R; Faculty of Medicine, Dalhousie University, Halifax, Nova Scotia, Canada., Gupta S; Faculty of Medicine, University of Ottawa, Ottawa, Ontario, Canada., Langille C; Faculty of Medicine, Division of Dermatology, Dalhousie University, Halifax, Nova Scotia, Canada., Sutherland A; Faculty of Medicine, Division of Dermatology, Dalhousie University, Halifax, Nova Scotia, Canada.
Πηγή: Pediatric dermatology [Pediatr Dermatol] 2026 May-Jun; Vol. 43 (3), pp. 579-589. Date of Electronic Publication: 2026 Feb 16.
Τύπος έκδοσης: Journal Article; Systematic Review
Γλώσσα: English
Στοιχεία περιοδικού: Publisher: Wiley Country of Publication: United States NLM ID: 8406799 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1525-1470 (Electronic) Linking ISSN: 07368046 NLM ISO Abbreviation: Pediatr Dermatol Subsets: MEDLINE
Imprint Name(s): Publication: <2010->: Hoboken, NJ : Wiley
Original Publication: [Boston, MA] : Blackwell Scientific Publications, [1983-
Ιατρικοί όροι (MeSH): Stevens-Johnson Syndrome*/diagnosis , Stevens-Johnson Syndrome*/therapy , Consensus Statements as Topic* , Practice Guidelines as Topic*, Child ; Humans ; Consensus
Περίληψη: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, life-threatening mucocutaneous reactions. Pediatric mortality is lower than in adults, but children face higher risks of recurrence and long-term sequelae. Despite disease severity, pediatric-specific guidelines remain scarce and often extrapolated from adult populations. We systematically reviewed and appraised national and international clinical practice guidelines and consensus statements on pediatric SJS/TEN, focusing on supportive care, pharmacologic interventions, mucosal management, and long-term follow-up. A systematic search of MEDLINE, Embase, PubMed, CENTRAL, Scopus, and CINAHL (January 2000-April 2025) identified national and international guidelines. For feasibility, we restricted full appraisal to documents published in English or French. Eligible documents provided explicit recommendations for patients < 18 years. Two reviewers independently extracted data and assessed quality using the AGREE II instrument. Of 144 records screened, 10 guidelines met inclusion criteria; 60% were published within the last 5 years, and 50% were consensus-based without systematic evidence synthesis. Most (90%) endorsed supportive measures such as wound care, nutritional support, and fluid resuscitation. Recommendations for pharmacologic therapy varied considerably: intravenous immunoglobulin (50%), systemic corticosteroids (40%), and cyclosporine (40%), with limited pediatric dosing guidance. Only one guideline mentioned biologics. Multidisciplinary care was recommended in 90%, yet long-term follow-up for sequelae appeared in only 50%. AGREE II appraisal showed high clarity of presentation but consistently low applicability; only the British Association of Dermatologists guideline achieved high quality across all domains. Current pediatric SJS/TEN guidelines display heterogeneity in scope and rigor. While supportive care is emphasized, pharmacologic and long-term management guidance remains inconsistent. Standardized, evidence-based, pediatric-specific guidelines are urgently needed.
(© 2026 The Author(s). Pediatric Dermatology published by Wiley Periodicals LLC.)
References: J Eur Acad Dermatol Venereol. 2016 Oct;30(10):1657-1669. (PMID: 27406069)
Ther Adv Chronic Dis. 2020 Apr 28;11:2040622319894469. (PMID: 32523661)
J Burn Care Res. 2009 May-Jun;30(3):437-49. (PMID: 19349890)
Int J Dermatol. 2022 Feb;61(2):158-163. (PMID: 34037244)
Acta Biomed. 2019 Jan 29;90(3-S):52-60. (PMID: 30830062)
Br J Dermatol. 2021 Aug;185(2):447-449. (PMID: 33730370)
Allergol Int. 2016 Jan;65(1):74-81. (PMID: 26666483)
Ophthalmol Ther. 2023 Aug;12(4):1795-1811. (PMID: 37140876)
JAMA Dermatol. 2021 Jun 01;157(6):712-715. (PMID: 33950191)
J Am Acad Dermatol. 2002 Oct;47(4):548-52. (PMID: 12271299)
Indian J Dermatol Venereol Leprol. 2016 Nov-Dec;82(6):603-625. (PMID: 27716721)
J Am Acad Dermatol. 2013 Sep;69(3):496-8. (PMID: 23957982)
JAMA Dermatol. 2024 Jan 1;160(1):117. (PMID: 38055233)
Dermatol Ther (Heidelb). 2023 Jun;13(6):1305-1327. (PMID: 37178320)
J Dtsch Dermatol Ges. 2024 Oct;22(10):1448-1466. (PMID: 39314017)
Br J Dermatol. 2017 Oct;177(4):924-935. (PMID: 28144971)
J Dtsch Dermatol Ges. 2024 Nov;22(11):1576-1593. (PMID: 39417754)
AACN Adv Crit Care. 2020 Sep 15;31(3):281-295. (PMID: 32866260)
J Clin Aesthet Dermatol. 2009 Mar;2(3):51-5. (PMID: 20729944)
Ann Dermatol Venereol. 2024 Jun;151(2):103282. (PMID: 38810539)
Br J Dermatol. 2019 Jul;181(1):37-54. (PMID: 30829411)
Int J Dermatol. 2023 Mar;62(3):397-403. (PMID: 36562634)
Plast Reconstr Surg. 1998 Sep;102(4):1018-22. (PMID: 9734418)
J Am Acad Dermatol. 2020 Jun;82(6):1553-1567. (PMID: 32151629)
Contributed Indexing: Keywords: SJS/TEN; clinical practice guideline; consensus statement; management; pediatrics; systematic review
Entry Date(s): Date Created: 20260216 Date Completed: 20260708 Latest Revision: 20260813
Update Code: 20260813
PubMed Central ID: PMC13206584
DOI: 10.1111/pde.70149
PMID: 41698348
Βάση Δεδομένων: MEDLINE
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  Data: Diagnosis and Management of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Pediatric Patients: A Systematic Review of Clinical Guidelines and Consensus Statements.
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  Data: &lt;searchLink fieldCode=&quot;AU&quot; term=&quot;%22Creighton+R%22&quot;&gt;Creighton R&lt;/searchLink&gt;; Faculty of Medicine, Dalhousie University, Halifax, Nova Scotia, Canada.&lt;br /&gt;&lt;searchLink fieldCode=&quot;AU&quot; term=&quot;%22Gupta+S%22&quot;&gt;Gupta S&lt;/searchLink&gt;; Faculty of Medicine, University of Ottawa, Ottawa, Ontario, Canada.&lt;br /&gt;&lt;searchLink fieldCode=&quot;AU&quot; term=&quot;%22Langille+C%22&quot;&gt;Langille C&lt;/searchLink&gt;; Faculty of Medicine, Division of Dermatology, Dalhousie University, Halifax, Nova Scotia, Canada.&lt;br /&gt;&lt;searchLink fieldCode=&quot;AU&quot; term=&quot;%22Sutherland+A%22&quot;&gt;Sutherland A&lt;/searchLink&gt;; Faculty of Medicine, Division of Dermatology, Dalhousie University, Halifax, Nova Scotia, Canada.
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  Data: &lt;searchLink fieldCode=&quot;JN&quot; term=&quot;%228406799%22&quot;&gt;Pediatric dermatology&lt;/searchLink&gt; [Pediatr Dermatol] 2026 May-Jun; Vol. 43 (3), pp. 579-589. &lt;i&gt;Date of Electronic Publication: &lt;/i&gt;2026 Feb 16.
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  Data: &lt;searchLink fieldCode=&quot;MM&quot; term=&quot;%22Stevens-Johnson+Syndrome%22&quot;&gt;Stevens-Johnson Syndrome*&lt;/searchLink&gt;/&lt;searchLink fieldCode=&quot;MM&quot; term=&quot;%22Stevens-Johnson+Syndrome+diagnosis%22&quot;&gt;diagnosis&lt;/searchLink&gt; &lt;br /&gt;&lt;searchLink fieldCode=&quot;MM&quot; term=&quot;%22Stevens-Johnson+Syndrome%22&quot;&gt;Stevens-Johnson Syndrome*&lt;/searchLink&gt;/&lt;searchLink fieldCode=&quot;MM&quot; term=&quot;%22Stevens-Johnson+Syndrome+therapy%22&quot;&gt;therapy&lt;/searchLink&gt; &lt;br /&gt;&lt;searchLink fieldCode=&quot;MM&quot; term=&quot;%22Consensus+Statements+as+Topic%22&quot;&gt;Consensus Statements as Topic*&lt;/searchLink&gt; &lt;br /&gt;&lt;searchLink fieldCode=&quot;MM&quot; term=&quot;%22Practice+Guidelines+as+Topic%22&quot;&gt;Practice Guidelines as Topic*&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;MH&quot; term=&quot;%22Child%22&quot;&gt;Child&lt;/searchLink&gt; ; &lt;searchLink fieldCode=&quot;MH&quot; term=&quot;%22Humans%22&quot;&gt;Humans&lt;/searchLink&gt; ; &lt;searchLink fieldCode=&quot;MH&quot; term=&quot;%22Consensus%22&quot;&gt;Consensus&lt;/searchLink&gt;
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  Data: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, life-threatening mucocutaneous reactions. Pediatric mortality is lower than in adults, but children face higher risks of recurrence and long-term sequelae. Despite disease severity, pediatric-specific guidelines remain scarce and often extrapolated from adult populations. We systematically reviewed and appraised national and international clinical practice guidelines and consensus statements on pediatric SJS/TEN, focusing on supportive care, pharmacologic interventions, mucosal management, and long-term follow-up. A systematic search of MEDLINE, Embase, PubMed, CENTRAL, Scopus, and CINAHL (January 2000-April 2025) identified national and international guidelines. For feasibility, we restricted full appraisal to documents published in English or French. Eligible documents provided explicit recommendations for patients &amp;lt; 18 years. Two reviewers independently extracted data and assessed quality using the AGREE II instrument. Of 144 records screened, 10 guidelines met inclusion criteria; 60% were published within the last 5 years, and 50% were consensus-based without systematic evidence synthesis. Most (90%) endorsed supportive measures such as wound care, nutritional support, and fluid resuscitation. Recommendations for pharmacologic therapy varied considerably: intravenous immunoglobulin (50%), systemic corticosteroids (40%), and cyclosporine (40%), with limited pediatric dosing guidance. Only one guideline mentioned biologics. Multidisciplinary care was recommended in 90%, yet long-term follow-up for sequelae appeared in only 50%. AGREE II appraisal showed high clarity of presentation but consistently low applicability; only the British Association of Dermatologists guideline achieved high quality across all domains. Current pediatric SJS/TEN guidelines display heterogeneity in scope and rigor. While supportive care is emphasized, pharmacologic and long-term management guidance remains inconsistent. Standardized, evidence-based, pediatric-specific guidelines are urgently needed.&lt;br /&gt; (&#169; 2026 The Author(s). Pediatric Dermatology published by Wiley Periodicals LLC.)
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  Data: J Eur Acad Dermatol Venereol. 2016 Oct;30(10):1657-1669. (PMID: &lt;searchLink fieldCode=&quot;PM&quot; term=&quot;%2227406069%22&quot;&gt;27406069)&lt;/searchLink&gt;&lt;br /&gt;Ther Adv Chronic Dis. 2020 Apr 28;11:2040622319894469. (PMID: &lt;searchLink fieldCode=&quot;PM&quot; term=&quot;%2232523661%22&quot;&gt;32523661)&lt;/searchLink&gt;&lt;br /&gt;J Burn Care Res. 2009 May-Jun;30(3):437-49. (PMID: &lt;searchLink fieldCode=&quot;PM&quot; term=&quot;%2219349890%22&quot;&gt;19349890)&lt;/searchLink&gt;&lt;br /&gt;Int J Dermatol. 2022 Feb;61(2):158-163. (PMID: &lt;searchLink fieldCode=&quot;PM&quot; term=&quot;%2234037244%22&quot;&gt;34037244)&lt;/searchLink&gt;&lt;br /&gt;Acta Biomed. 2019 Jan 29;90(3-S):52-60. (PMID: &lt;searchLink fieldCode=&quot;PM&quot; term=&quot;%2230830062%22&quot;&gt;30830062)&lt;/searchLink&gt;&lt;br /&gt;Br J Dermatol. 2021 Aug;185(2):447-449. (PMID: &lt;searchLink fieldCode=&quot;PM&quot; term=&quot;%2233730370%22&quot;&gt;33730370)&lt;/searchLink&gt;&lt;br /&gt;Allergol Int. 2016 Jan;65(1):74-81. (PMID: &lt;searchLink fieldCode=&quot;PM&quot; term=&quot;%2226666483%22&quot;&gt;26666483)&lt;/searchLink&gt;&lt;br /&gt;Ophthalmol Ther. 2023 Aug;12(4):1795-1811. (PMID: &lt;searchLink fieldCode=&quot;PM&quot; term=&quot;%2237140876%22&quot;&gt;37140876)&lt;/searchLink&gt;&lt;br /&gt;JAMA Dermatol. 2021 Jun 01;157(6):712-715. (PMID: &lt;searchLink fieldCode=&quot;PM&quot; term=&quot;%2233950191%22&quot;&gt;33950191)&lt;/searchLink&gt;&lt;br /&gt;J Am Acad Dermatol. 2002 Oct;47(4):548-52. (PMID: &lt;searchLink fieldCode=&quot;PM&quot; term=&quot;%2212271299%22&quot;&gt;12271299)&lt;/searchLink&gt;&lt;br /&gt;Indian J Dermatol Venereol Leprol. 2016 Nov-Dec;82(6):603-625. (PMID: &lt;searchLink fieldCode=&quot;PM&quot; term=&quot;%2227716721%22&quot;&gt;27716721)&lt;/searchLink&gt;&lt;br /&gt;J Am Acad Dermatol. 2013 Sep;69(3):496-8. (PMID: &lt;searchLink fieldCode=&quot;PM&quot; term=&quot;%2223957982%22&quot;&gt;23957982)&lt;/searchLink&gt;&lt;br /&gt;JAMA Dermatol. 2024 Jan 1;160(1):117. (PMID: &lt;searchLink fieldCode=&quot;PM&quot; term=&quot;%2238055233%22&quot;&gt;38055233)&lt;/searchLink&gt;&lt;br /&gt;Dermatol Ther (Heidelb). 2023 Jun;13(6):1305-1327. (PMID: &lt;searchLink fieldCode=&quot;PM&quot; term=&quot;%2237178320%22&quot;&gt;37178320)&lt;/searchLink&gt;&lt;br /&gt;J Dtsch Dermatol Ges. 2024 Oct;22(10):1448-1466. (PMID: &lt;searchLink fieldCode=&quot;PM&quot; term=&quot;%2239314017%22&quot;&gt;39314017)&lt;/searchLink&gt;&lt;br /&gt;Br J Dermatol. 2017 Oct;177(4):924-935. (PMID: &lt;searchLink fieldCode=&quot;PM&quot; term=&quot;%2228144971%22&quot;&gt;28144971)&lt;/searchLink&gt;&lt;br /&gt;J Dtsch Dermatol Ges. 2024 Nov;22(11):1576-1593. (PMID: &lt;searchLink fieldCode=&quot;PM&quot; term=&quot;%2239417754%22&quot;&gt;39417754)&lt;/searchLink&gt;&lt;br /&gt;AACN Adv Crit Care. 2020 Sep 15;31(3):281-295. (PMID: &lt;searchLink fieldCode=&quot;PM&quot; term=&quot;%2232866260%22&quot;&gt;32866260)&lt;/searchLink&gt;&lt;br /&gt;J Clin Aesthet Dermatol. 2009 Mar;2(3):51-5. (PMID: &lt;searchLink fieldCode=&quot;PM&quot; term=&quot;%2220729944%22&quot;&gt;20729944)&lt;/searchLink&gt;&lt;br /&gt;Ann Dermatol Venereol. 2024 Jun;151(2):103282. (PMID: &lt;searchLink fieldCode=&quot;PM&quot; term=&quot;%2238810539%22&quot;&gt;38810539)&lt;/searchLink&gt;&lt;br /&gt;Br J Dermatol. 2019 Jul;181(1):37-54. (PMID: &lt;searchLink fieldCode=&quot;PM&quot; term=&quot;%2230829411%22&quot;&gt;30829411)&lt;/searchLink&gt;&lt;br /&gt;Int J Dermatol. 2023 Mar;62(3):397-403. (PMID: &lt;searchLink fieldCode=&quot;PM&quot; term=&quot;%2236562634%22&quot;&gt;36562634)&lt;/searchLink&gt;&lt;br /&gt;Plast Reconstr Surg. 1998 Sep;102(4):1018-22. (PMID: &lt;searchLink fieldCode=&quot;PM&quot; term=&quot;%229734418%22&quot;&gt;9734418)&lt;/searchLink&gt;&lt;br /&gt;J Am Acad Dermatol. 2020 Jun;82(6):1553-1567. (PMID: &lt;searchLink fieldCode=&quot;PM&quot; term=&quot;%2232151629%22&quot;&gt;32151629)&lt;/searchLink&gt;
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