Introduction: Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, life-threatening, allergic reactions affecting the skin and mucous membranes. SJS is considered to be a milder form with less than 10% of body surface area (BSA) involvement. We report successful management of two cases of SJS and TEN. Firstly, a case of a 24-year-old female who presented with rashes over face, chest, and upper limbs after the oral intake of ciprofloxacin and local application of moxifloxacin eye drops. She developed high-grade fever and difficulty in breathing requiring intubation and lung-protective mechanical ventilation and was treated with high-dose methylprednisolone, azithromycin, soframycin skin dressings, and topical ocular antibiotics. Secondly, another case of a 16-year-old female who developed bullous eruptions over the trunk, arms, hands, face, and sole involving 60% of BSA, after oral intake of albendazole. She was diagnosed as TEN and successfully managed with sterile silver nitrate, soframycin dressings, and antibiotics.
Key message: Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening, allergic reactions affecting the skin and mucous membranes. Early identification, withdrawal of the suspected drug, and early transfer to a specialized center decrease mortality.
Bastuji-Garin S, Rzany B, Stern RS, Shear NH, Naldi L, Roujeau JC. Clinical classification of cases of toxic epidermal necrolysis, Stevens- Johnson syndrome and erythema multiforme. Arch Dermatol 1993;129(1):92–96. DOI: 10.1001/archderme.
Barvaliya M, Sanmukhani J, Patel T, Paliwal N, Shah H, Tripathi C. Drug-induced Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and SJS-TEN overlap: a multicentric retrospective study. J Postgrad Med 2011;57(2):115–119. DOI: 10.4103/0022-3859.81865.
Roujeau JC, Kelly JP, Naldi L, Rzany B, Stern RS, Anderson T, 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.
Sethuraman G, Sharma VK, Pahwa P, Khetan P. Causative drugs and clinical outcome in Stevens Johnson Syndrome (SJS), Toxic Epidermal Necrolysis (TEN), and SJS-TEN overlap in children. Indian J Dermatol 2012;57(3):199–200. DOI: 10.4103/0019-5154.96192.
Mockenhaupt M, Viboud C, Dunant A, Naldi L, Halevy S, Bouwes Bavinck JN, 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.
Mulvey JM, Padowitz A, Lindley-Jones M, Nickels R. Mycoplasma pneumoniae associated with Stevens Johnson syndrome. Anaesth Intensive Care 2007;35(3):414–417. DOI: 10.1177/0310057X0703500317.
Forman R, Koren G, Shear NH. Erythema multiforme, Stevens-Johnson syndrome and toxic epidermal necrolysis in children: a review of 10 years’ experience. Drug Saf 2002;25(13):965–972. DOI: 10.2165/00002018-200225130-00006.
Zakrzewski JL, Lentini G, Such U, Duerr A, Tran V, Guenzelmann S, et al. Toxic epidermal necrolysis: differential diagnosis of an epidermolytic dermopathy in a hematopoietic stem cell transplant recipient. Bone Marrow Transplant 2002;30(5):331–333. DOI: 10.1038/sj.bmt.1703624.
Katoulis AC, Liakou A, Bozi E, Theodorakis M, Alevizou A, Zafeiraki A, et al. Erythema multiforme following vaccination for human papillomavirus. Dermatology 2010;220(1):60–62. DOI: 10.1159/000254898.
Abe R. Immunological response in Stevens-Johnson syndrome and toxic epidermal necrolysis. J Dermatol 2015;42(1):42–48. DOI: 10.1111/1346-8138.12674.
Teraki Y, Shibuya M, Izaki S. Stevens-Johnson syndrome and toxic epidermal necrolysis due to anticonvulsants share certain clinical and laboratory features with drug-induced hypersensitivity syndrome, despite differences in cutaneous presentations. Clin Exp Dermatol 2010;35(7):723–728. DOI: 10.1111/j.1365-2230.2009.03718.x.
López-García JS, Rivas Jara L, García-Lozano CI, Conesa E, de Juan IE, Murube del Castillo J. Ocular features and histopathologic changes during follow-up of toxic epidermal necrolysis. Ophthalmology 2011;118(2):265–271. DOI: 10.1016/j.ophtha.2010.06.035.
Sotozono C, Ueta M, Koizumi N, Inatomi T, Shirakata Y, Ikezawa Z, et al. Diagnosis and treatment of Stevens-Johnson syndrome and toxic epidermal necrolysis with ocular complications. Ophthalmology 2009;116(4):685–690. DOI: 10.1016/j.ophtha.2008.12.048.
Gueudry J, Roujeau JC, Binaghi M, Soubrane G, Muraine M. Risk factors for the development of ocular complications of Stevens- Johnson syndrome and toxic epidermal necrolysis. Arch Dermatol 2009;145(2):157–162. DOI: 10.1001/archdermatol.2009.540.
Hwang SH, Kim DH, Kim JH, Son BK, Lim DH. Chronic pulmonary complications due to toxic epidermal necrolysis. Allergy Asthma Respir Dis 2013;1(4):391–394. DOI: 10.4168/aard.2013.1.4.391.
Kamada N, Kinoshita K, Togawa Y, Kobayashi T, Matsubara H, Kohno M, et al. Chronic pulmonary complications associated with toxic epidermal necrolysis: report of a severe case with anti-Ro/SS-A and a review of the published work. J Dermatol 2006;33(9):616–622. DOI: 10.1111/j.1346-8138.2006.00142.x.
Kaser DJ, Reichman DE, Laufer MR. Prevention of vulvovaginal sequelae in Stevens-Johnson syndrome and toxic epidermal necrolysis. Rev Obstet Gynecol 2011;4(2):81–85. DOI: 10.3909/riog0152.
Oplatek A, Brown K, Sen S, Halerz M, Supple K, Gamelli RL. Long-term follow-up of patients treated for toxic epidermal necrolysis. J Burn Care Res 2006;27(1):26–33. DOI: 10.1097/01.bcr.0000194268.01514.f8.
Guégan S, Bastuji-Garin S, Poszepczynska-Guigné E, Roujeau JC, Revuz J. Performance of the SCORTEN during the first 5 days of hospitalization to predict the prognosis of epidermal necrolysis. J Invest Dermatol 2006;126(2):272–276. DOI: 10.1038/sj.jid.5700068.
Ramam M, Kumar U, Bhat R, Sharma VK. Oral drug provocation test to generate a list of safe drugs: experience with 100 patients. Indian J Dermatol Venereol Leprol 2012;78(5):595–598. DOI: 10.4103/0378-6323.100563.
Garcia-Doval I, LeCleach L, Bocquet H, Otero XL, Roujeau JC. Toxic epidermal necrolysis and Stevens-Johnson syndrome: does early withdrawal of causative drugs decrease the risk of death? Arch Dermatol 2000;136(3):323–327. DOI: 10.1001/archderm.136.3.323.
McGee T, Munster A. Toxic epidermal necrolysis syndrome: mortality rate reduced with early referral to regional burn center. Plast Reconstr Surg 1998;102(4):1018–1022. DOI: 10.1097/00006534-199809040-00014.
Palmieri TL, Greenhalgh DG, Saffle JR, Spence RJ, Peck MD, Jeng JC, et al. A multicenter review of toxic epidermal necrolysis treated in U.S. burn centers at the end of the twentieth century. J Burn Care Rehabil 2002;23(2):87–96. DOI: 10.1097/00004630-200203000-00004.
Worswick S, Cotliar J. Stevens–Johnson syndrome and toxic epidermal necrolysis: a review of treatment options. Dermatol Ther 2011;24(2):207–218. DOI: 10.1111/j.1529-8019.2011.01396.x.
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.
Ramakrishnan KM, Sankar J, Venkatraman J. Role of biological membranes in the management of Stevens Johnson syndrome – Indian experience. Burns 2007;33(1):109–111. DOI: 10.1016/j.burns.2006.04.029.
Li X, Wang D, Lu Z, Chen J, Zhang H, Sun L. Umbilical cord mesenchymal stem cell transplantation in drug-induced Stevens-Johnson syndrome. J Eur Acad Dermatol Venereol 2013;27(5):659–661. DOI: 10.1111/j.1468-3083.2012.04572.x.
Sharma N, Thenarasun SA, Kaur M, Pushker N, Khanna N, Agarwal T, et al. Adjuvant role of amniotic membrane transplantation in acute ocular Stevens-Johnson syndrome: a randomized control trial. Ophthalmology 2016;123(3):484–491. DOI: 10.1016/j.ophtha.2015.10.027.
O’Donoghue JM, Céspedes YP, Rockley PF, Nigra TP. Skin biopsies to assess response to systemic corticosteroid therapy in early-stage TEN: case report and review of the literature. Cutis 2009;84(3):138–140.
Hynes AY, Kafkala C, Daoud YJ, Foster CS. Controversy in the use of high-dose systemic steroids in the acute care of patients with Stevens-Johnson syndrome. Int Ophthalmol Clin 2005;45(4):25–48. DOI: 10.1097/01.iio.0000177430.89645.6d.
Prins C, Kerdel FA, Padilla RS, Hunziker T, Chimenti S, Viard I, et al. Treatment of toxic epidermal necrolysis with high dose intravenous immunoglobulins: multicenter retrospective analysis of 48 consecutive cases. Arch Dermatol 2003;139(1): 26–32. DOI: 10.1001/archderm.139.1.26.
Barron SJ, Del Vecchio MT, Aronoff SC. Intravenous immunoglobulin in the treatment of Stevens-Johnson syndrome and toxic epidermal necrolysis: a meta-analysis with meta-regression of observational studies. Int J Dermatol 2015;54(1):108–115. DOI: 10.1111/ijd.12423.
Bachot N, Revuz J, Roujeau JC. Intravenous immunoglobulin treatment for Stevens-Johnson syndrome and toxic epidermal necrolysis: a prospective noncomparative study showing no benefit on mortality or progression. Arch Dermatol 2003;139(1):33–36. DOI: 10.1001/archderm.139.1.33.
Chave TA, Mortimer NJ, Sladden MJ, Hall AP, Hutchinson PE. Toxic epidermal necrolysis: current evidence, practical management and future directions. Br J Dermatol 2005;153(2):241–253. DOI: 10.1111/j.1365-2133.2005.06721.x.
Singh GK, Chatterjee M, Verma R. Cyclosporine in Stevens Johnson syndrome and toxic epidermal necrolysis and retrospective comparison with systemic corticosteroid. Indian J Dermatol Venereol Leprol 2013;79(5):686–692. DOI: 10.4103/0378-6323.116738.
Kirchhof MG, Miliszewski MA, Sikora S, Papp A, Dutz JP. Retrospective review of Stevens-Johnson syndrome/toxic epidermal necrolysis treatment comparing intravenous immunoglobulin with cyclosporine. J Am Acad Dermatol 2014;71(5):941–947. DOI: 10.1016/j.jaad.2014.07.016.
Giudice G, Maggio G, Bufano L, Memeo G, Vestita M. Management of toxic epidermal necrolysis with plasmapheresis and cyclosporine A: our 10 years’ experience. Plast Reconstr Surg Glob Open 2017;5(2):e1221. DOI: 10.1097/GOX.0000000000001221.
Heng MC, Allen SG. Efficacy of cyclophosphamide in toxic epidermal necrolysis. Clinical and pathophysiologic aspects. J Am Acad Dermatol 1991;25(5 Pt 1):778–786. DOI: 10.1016/s0190-9622(08)80969-3.
Dogra PM, Chatterjee M, Neema S. Tacrolimus for treatment of toxic epidermal necrolysis. Indian J Dermatol Venereol Leprol 2015;81(6):642–644. DOI: 10.4103/0378-6323.168328.
Hunger RE, Hunziker T, Buettiker U, Braathen LR, Yawalkar N. Rapid resolution of toxic epidermal necrolysis with anti-TNF-alpha treatment. J Allergy Clin Immunol 2005;116(4):923–924. DOI: 10.1016/j.jaci.2005.06.029.
Meiss F, Helmbold P, Meykadeh N, Gaber G, Marsch WCh, Fischer M. Overlap of acute generalized exanthematous pustulosis and toxic epidermal necrolysis: response to antitumour necrosis factor-alpha antibody infliximab: report of three cases. J Eur Acad Dermatol Venereol 2007;21(5):717–719. DOI: 10.1111/j.1468-3083.2006.02026.x.
Egan CA, Grant WJ, Morris SE, Saffle JR, Zone JJ. Plasmapheresis as an adjunct treatment in toxic epidermal necrolysis. J Am Acad Dermatol 1999;40(3):458–461. DOI: 10.1016/s0190-9622(99)70497-4.
Furubacke A, Berlin G, Anderson C, Sjöberg F. Lack of significant treatment effect of plasma exchange in the treatment of drug-induced toxic epidermal necrolysis? Intensive Care Med 1999;25(11):1307–1310. DOI: 10.1007/s001340051063.
Su SC, Chung WH. Cytotoxic proteins and therapeutic targets in severe cutaneous adverse reactions. Toxins (Basel) 2014;6(1):194–210. DOI: 10.3390/toxins6010194.
Thong BY. Stevens-Johnson syndrome/toxic epidermal necrolysis: an Asia-Pacific perspective. Asia Pac Allergy 2013;3(4):215–223. DOI: 10.5415/apallergy.2013.3.4.215.