Keywords :
Emergency medicine, Muco-cutaneous disease, Pulse therapy, Toxic epidermal necrolysis, Severity-of-illness score for TEN
Citation Information :
Deivassigamany G, Ramanathan N, Ezhilan K. Dermatological Emergency in Emergency Medicine: A Case Series. Nat J Emerg Med 2023; 1 (3):71-76.
Toxic epidermal necrolysis (TEN), is an acute, potentially life-threatening mucocutaneous disease often induced by drugs due to an inappropriate immune reaction. It is characterized by the widespread detachment and exfoliation of the epidermis and mucous membrane, leaving large areas of raw, exposed tissue. It is a medical emergency and requires immediate treatment. The condition might result in sepsis, a deadly complication if not treated promptly. It involves >30% of the total body surface area (TBSA), which helps to differentiate it from Stevens-Johnson syndrome (SJS) which involves 10% of TBSA. It usually occurs within a few weeks of treatment initiation, and requires a detailed medication history that plays a pivotal role in the diagnosis. The severity-of-illness score for TEN (SCORTEN) is used to estimate the prognosis. In this report, we report three cases of drug-induced severe TEN who were presented to the emergency room (ER). The presence of the characteristic skin lesions led to the diagnosis of TEN in these cases. Their TBSA was >30%, with a positive Nikolsky sign. Two of the cases had a SCORTEN of 2, and one had a SCORTEN of 6 with a significant history. Treatment was initiated with the withdrawal of the potential agent and pulse therapy with conservative treatment.
Labib AM, Milroy C. Toxic Epidermal Necrolysis. In: StatPearls. Treasure Island (FL): StatPearls Publishing; 2022. Available from: http://www.ncbi.nlm.nih.gov/books/NBK574530/.
Stătescu L, Constantin M, Morariu HS, et al. Toxic epidermal necrolysis-A case report. J Crit Care Med Univ Med Si Farm Din Targu-Mures 2017;3(1):29–33. DOI: https://doi.org/10.1515/jccm-2017-0002.
Chaby G, Ingen-Housz-Oro S, De Prost N, et al. Idiopathic Stevens-Johnson syndrome and toxic epidermal necrolysis: Prevalence and patients’ characteristics. J Am Acad Dermatol 2019;80(5):1453–1455. DOI: 10.1016/j.jaad.2018.10.058.
Kim HI, Kim SW, Park GY, et al. Causes and treatment outcomes of Stevens-Johnson syndrome and toxic epidermal necrolysis in 82 adult patients. Korean J Intern Med 2012;27(2):203–210. DOI: 10.3904/kjim.2012.27.2.203.
Schwartz RA, McDonough PH, Lee BW. Toxic epidermal necrolysis: Part I. Introduction, history, classification, clinical features, systemic manifestations, etiology, and immunopathogenesis. J Am Acad Dermatol 2013;69(2):173.e1–e13; quiz 185–186. DOI: 10.1016/j.jaad.2013.05.003.
Hsu DY, Brieva J, Silverberg NB, et al. Morbidity and mortality of Stevens-Johnson syndrome and toxic epidermal necrolysis in United States adults. J Invest Dermatol 2016;136(7):1387–1397. DOI: 10.1016/j.jid.2016.03.023.
Poulsen VOB, Nielsen J, Poulsen TD. Rapidly developing toxic epidermal necrolysis. Case Rep Emerg Med 2013;2013:e985951. DOI: 10.1155/2013/985951.
Bastuji-Garin S, Rzany B, Stern RS, et al. Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme. Arch Dermatol 1993;129(1):92–96. PMID: 8420497.
Roujeau JC, Kelly JP, Naldi L, et al. Medication use and the risk of Stevens-Johnson syndrome or toxic epidermal necrolysis. N Engl J Med 1995;333(24):1600–1607. DOI: 10.1056/NEJM199512143332404.
Creamer D, Walsh SA, Dziewulski P, et al. U.K. guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in adults 2016. Br J Dermatol 2016;174(6):1194–1227. DOI: https://doi.org/10.1111/bjd.14530.
Lyell A. Toxic epidermal necrolysis: An eruption resembling scalding of the skin. Br J Dermatol 1956;68(11):355–361. DOI: 10.1111/j.1365-2133.1956.tb12766.x.
Mockenhaupt M, Viboud C, Dunant A, et al. Stevens-Johnson syndrome and toxic epidermal necrolysis: Assessment of medication risks with emphasis on recently marketed drugs. The EuroSCAR-study. J Invest Dermatol 2008;128(1):35–44. DOI: 10.1038/sj.jid.5701033.
Wu J, Lee YY, Su SC, et al. Stevens-Johnson syndrome and toxic epidermal necrolysis in patients with malignancies. Br J Dermatol 2015;173(5):1224–1231. DOI: 10.1111/bjd.14052.
Chahal D, Aleshin M, Turegano M, et al. Vaccine-induced toxic epidermal necrolysis: A case and systematic review. Dermatol Online J 2018;24(1):13030/qt7qn5268s. PMID: 29469759.
Paquet P, Piérard GE. Glutathione-S-transferase pi expression in toxic epidermal necrolysis: A marker of putative oxidative stress in keratinocytes. Skin Pharmacol Physiol 2007;20(2):66–70. DOI: 10.1159/000097652.
Hayes JD, Pulford DJ. The glutathione S-transferase supergene family: Regulation of GST and the contribution of the isoenzymes to cancer chemoprotection and drug resistance. Crit Rev Biochem Mol Biol 1995;30(6):445–600. DOI: 10.3109/10409239509083491.
Chung WH, Hung SI, Yang JY, et al. Granulysin is a key mediator for disseminated keratinocyte death in Stevens-Johnson syndrome and toxic epidermal necrolysis. Nat Med 2008;14(12):1343–1350. DOI: 10.1038/nm.1884.
Abe R. Toxic epidermal necrolysis and Stevens-Johnson syndrome: Soluble Fas ligand involvement in the pathomechanisms of these diseases. J Dermatol Sci 2008;52(3):151–159. DOI: 10.1016/j.jdermsci.2008.06.003.
Sassolas B, Haddad C, Mockenhaupt M, et al. ALDEN, an algorithm for assessment of drug causality in Stevens-Johnson syndrome and toxic epidermal necrolysis: Comparison with case-control analysis. Clin Pharmacol Ther 2010;88(1):60–68. DOI: 10.1038/clpt.2009.252.
Harr T, French LE. Toxic epidermal necrolysis and Stevens-Johnson syndrome. Orphanet J Rare Dis 2010;5:39. DOI: 10.1186/1750-1172- 5-39.
Fouchard N, Bertocchi M, Roujeau JC, et al. SCORTEN: A severity-of-illness score for toxic epidermal necrolysis. J Invest Dermatol 2000;115(2):149–153. DOI: 10.1046/j.1523-1747.2000.00061.x.
Sharma VK, Sethuraman G, Kumar B. Cutaneous adverse drug reactions: Clinical pattern and causative agents–a 6-year series from Chandigarh, India. J Postgrad Med 2001;47(2):95–99. PMID: 11832597.
Aihara M, Kano Y, Fujita H, et al. Efficacy of additional I.V. immunoglobulin to steroid therapy in Stevens-Johnson syndrome and toxic epidermal necrolysis. J Dermatol 2015;42(8):768–777. DOI: 10.1111/1346-8138.12925.
Mittal R, Sudha R, Murugan S, et al. Pulse therapy in dermatology. Sri Ramachandra J Med 2007;1(2):44–46. Available from: https://www.sriramachandra.edu.in/university/pdf/research/journals/jan_2007/book_9.pdf.
Seminario-Vidal L, Kroshinsky D, Malachowski SJ, et al. Society of dermatology hospitalists supportive care guidelines for the management of stevens-johnson syndrome/toxic epidermal necrolysis in adults. J Am Acad Dermatol 2020;82(6):1553–1567. DOI: 10.1016/j.jaad.2020.02.066.