Spektrum Sindrom Stevens–Johnson dan Nekrolisis Epidermal Toksik: Tinjauan Patogenesis, Diagnosis, dan Tatalaksana

Intan Wuri Handayani; Lusiana Lusiana; Hadi Firmansyah

doi:10.55606/jurrike.v5i1.8082

Authors

Intan Wuri Handayani Universitas YARSI
Lusiana Lusiana Universitas YARSI
Hadi Firmansyah Universitas YARSI

DOI:

https://doi.org/10.55606/jurrike.v5i1.8082

Keywords:

Drug Hypersensitivity, Epidermal Necrolysis, SCORTEN, Stevens–Johnson Syndrome, Toxic Epidermal Necrolysis

Abstract

Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, life-threatening mucocutaneous reactions characterized by extensive epidermal necrolysis and mucosal involvement. The entities represent a clinical continuum distinguished by the extent of epidermal detachment (SJS <10% body surface area [BSA], SJS/TEN overlap 10–30%, TEN >30%). Most cases are drug-induced, making early culprit-drug withdrawal and structured supportive care the cornerstones of management. Immunopathogenesis is primarily mediated by drug-specific cytotoxic T cells and NK cells through granulysin, Fas–Fas ligand, and perforin/granzyme pathways, with emerging roles of necroptosis and candidate biomarkers such as RIP3 and galectin-7. Diagnosis relies on timely recognition of the characteristic painful erythematous/targetoid lesions, Nikolsky/Asboe–Hansen signs, multi-site mucositis, accurate BSA assessment, and early severity scoring (e.g., SCORTEN) to guide referral and monitoring. Systemic immunomodulators (corticosteroids, cyclosporine, IVIg, anti-TNF agents) have variable evidence; thus, individualized selection and early multidisciplinary care particularly ophthalmologic involvement are essential to reduce acute mortality and long-term sequelae.

Downloads

Download data is not yet available.

References

Acar, A., Yoldaş, A.H., Türk, B.G., Karaarslan, I., Sağduyu, İ.E., Ceylan, C., Ünal, İ. and Öztürk, G. (2022) Stevens–Johnson syndrome and toxic epidermal necrolysis: 11-year demographic clinical and prognostic characteristics. Indian Journal of Dermatology, 67(1), pp. 12–18. https://doi.org/10.4103/ijd.IJD_671_21

Chang, H., Chung, W-H., & Hung, S-I. (2022). Stevens–Johnson syndrome and toxic epidermal necrolysis: Pathogenesis, diagnosis, and therapeutic strategies. Allergy, Asthma & Immunology Research, 14(1), 1–16.

Frantz, R., Mockenhaupt, M., & Dunant, A. (2021). Clinical recognition and management of Stevens–Johnson syndrome and toxic epidermal necrolysis. Orphanet Journal of Rare Diseases, 16(1), 1–15.

Hasegawa, A., & Abe, R. (2024). Recent advances in the immunopathogenesis of Stevens–Johnson syndrome and toxic epidermal necrolysis. Journal of Dermatological Science, 113(4), 2294–2303.

Heuer, M., Mockenhaupt, M., & Paulmann, M. (2024). S3 guideline: Stevens–Johnson syndrome and toxic epidermal necrolysis (epidermal necrolysis). Journal der Deutschen Dermatologischen Gesellschaft, 22(11), 1448–1456.

Justice, J., Mukherjee, E., Martin-Pozo, M. and Phillips, E.J. (2025) Updates in the pathogenesis of Stevens–Johnson syndrome and toxic epidermal necrolysis. Allergology International, 74(3), pp. 361–371. https://doi.org/10.1016/j.alit.2025.05.002

Kaimal, S., Lobo, C., Narayan, G. and Augustine, M. (2023) Stevens–Johnson syndrome and toxic epidermal necrolysis: A fresh look at an old foe. Indian Journal of Dermatology, 68(1), pp. 34–40. https://doi.org/10.4103/ijd.ijd_726_22

Menaldi, S. L. S. W., Bramono, K., & Indriatmi, W. (Eds.). (2016). Ilmu penyakit kulit dan kelamin (Edisi ke-7). Jakarta: Badan Penerbit FKUI.

Mockenhaupt, M., & Roujeau, J-C. (2019). Epidermal necrolysis (Stevens–Johnson syndrome and toxic epidermal necrolysis). Dalam S. Kang dkk. (Eds.), Fitzpatrick’s Dermatology (Edisi ke-9, hlm. 733–748). New York: McGraw-Hill.

Mukherjee, E., Phillips, E.J. and Dodiuk-Gad, R.P. (2024) Update on Stevens–Johnson syndrome and toxic epidermal necrolysis: Diagnosis and management. American Journal of Clinical Dermatology, 25, pp. 891–908. https://doi.org/10.1007/s40257-024-00889-6

Paulmann, M., dkk. (2024). S3 guideline: Diagnosis and treatment of epidermal necrolysis (Stevens–Johnson syndrome and toxic epidermal necrolysis) – Part 2: Supportive therapy of EN in the acute and post-acute stages. Journal der Deutschen Dermatologischen Gesellschaft (JDDG), 22, 1576–1593.

PERDOSKI. (2024). Pedoman nasional pelayanan kedokteran: Sindrom Stevens–Johnson dan nekrolisis epidermal toksik. Jakarta: Perhimpunan Dokter Spesialis Kulit dan Kelamin Indonesia.

Phillips, E. J., Bouchard, C. S., & Divito, S. J. (2022). Stevens–Johnson syndrome and toxic epidermal necrolysis: Coordinating research priorities to move the field forward. JAMA Dermatology, 158(6), 607–608.

Shah, H., Parisi, R., Mukherjee, E., Phillips, E.J. and Dodiuk-Gad, R.P. (2024) Update on Stevens–Johnson syndrome and toxic epidermal necrolysis: Diagnosis and management. American Journal of Clinical Dermatology, 25, pp. 891–908. https://doi.org/10.1007/s40257-024-00889-6

Sugito, T., Putera, I., & Damayanti, D. (2024). Classification and clinical features of epidermal necrolysis. Indonesian Journal of Dermatology and Venereology, 56(8), 1198–1199.

Sutedja, E., Rahmawati, D., & Hidayat, A. (2025). Clinical characteristics and causative drugs of Stevens–Johnson syndrome/toxic epidermal necrolysis in Bandung. Indonesian Journal of Dermatology and Venereology, 57(9), 979–981.Shah, H., Parisi, R.,