Paeds Vivas · paediatric-dermatology
Stevens-Johnson syndrome and toxic epidermal necrolysis — branching viva
Branching structured-oral viva on Stevens-Johnson syndrome and toxic epidermal necrolysis: the SJS, overlap and TEN classification by body-surface-area detachment, the drug-specific CD8-positive T-cell and granulysin pathophysiology, the anticonvulsant, sulfonamide, allopurinol and nevirapine culprits and the HLA-B15:02 and HLA-B58:01 pharmacogenetics, SCORTEN, the distinction from staphylococcal scalded skin syndrome and Mycoplasma-induced rash and mucositis, and the supportive-care-centred management with the no-proven-benefit position on immunomodulation.
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Opening question
Examiner: Take me through this child. What is the diagnosis, and what is your immediate frame? [6]
Candidate: This is Stevens-Johnson syndrome-toxic epidermal necrolysis overlap, a severe drug-induced mucocutaneous reaction. The keys are the carbamazepine started six weeks ago, the atypical target lesions, the sheet-like detachment of about 12 percent of body surface area, the positive Nikolsky sign, and the mucositis at two or more sites — the eyes and the mouth. My immediate frame is three things: stop the culprit drug at once, grade the severity with SCORTEN and admit to a burn-capable setting, and involve ophthalmology within hours to protect the eye. [6] [1]
Examiner: How do you classify this spectrum? [1]
Candidate: By the percentage of body-surface-area with epidermal detachment. Stevens-Johnson syndrome is under 10 percent, SJS-TEN overlap is 10 to 30 percent, and toxic epidermal necrolysis is more than 30 percent. All three share atypical target lesions, a positive Nikolsky sign and mucositis at two or more sites. This child, at 12 percent, is in the overlap band. [1]
References8ShowHide
- [1]Bastuji-Garin S; Fouchard N; Bertocchi M; Roujeau JC; et al SCORTEN: a severity-of-illness score for toxic epidermal necrolysis. J Invest Dermatol, 2000.PMID 10951229
- [3]Lonjou C; Borot N; Sekula P; Ledger N; et al A European study of HLA-B in Stevens-Johnson syndrome and toxic epidermal necrolysis related to five high-risk drugs. Pharmacogenet Genomics, 2008.PMID 18192896
- [4]Chung WH; Hung SI; Hong HS; Hsih MS; et al Medical genetics: a marker for Stevens-Johnson syndrome. Nature, 2004.PMID 15057820
- [5]Canavan TN; Mathes EF; Frieden I; Shinkai K Mycoplasma pneumoniae-induced rash and mucositis as a syndrome distinct from Stevens-Johnson syndrome and erythema multiforme: a systematic review. J Am Acad Dermatol, 2015.PMID 25592340
- [6]Halevy S; Ghislain PD; Mockenhaupt M; Fagot JP; et al Allopurinol is the most common cause of Stevens-Johnson syndrome and toxic epidermal necrolysis in Europe and Israel. J Am Acad Dermatol, 2008.PMID 17919772
- [9]Zimmermann S; Sekula P; Venhoff M; Motschall E; et al Systemic Immunomodulating Therapies for Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Systematic Review and Meta-analysis. JAMA Dermatol, 2017.PMID 28329382
- [10]AlFada M; Alotaibi H; Alsharif S; Alani AH; et al Systematic review, methodological appraisal, and recommendation mapping of clinical practice guidelines for managing patients with Stevens-Johnson syndrome and toxic epidermal necrolysis. J Dermatolog Treat, 2025.PMID 40010698
- [12]Chung WH; Hung SI Recent advances in the genetics and immunology of Stevens-Johnson syndrome and toxic epidermal necrosis. J Dermatol Sci, 2012.PMID 22541332