Clinical characteristics and treatment outcome of Stevens-Johnson syndrome and toxic epidermal necrolysis

Chantaphakul,Hiroshi; Sanon,Thanomsak; Klaewsongkram,Jettanong

doi:10.3892/etm.2015.2549

Clinical characteristics and treatment outcome of Stevens-Johnson syndrome and toxic epidermal necrolysis

Authors:
- Hiroshi Chantaphakul
- Thanomsak Sanon
- Jettanong Klaewsongkram
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Affiliations: Department of Medicine, Division of Allergy and Immunology, Faculty of Medicine, Chulalongkorn University, Patumwan, Bangkok 10330, Thailand
Published online on: June 5, 2015 https://doi.org/10.3892/etm.2015.2549
Pages: 519-524

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Abstract

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are erythematous skin lesions with blister formation accompanied by mucosal involvement. These conditions are considered to be life‑threatening illnesses. Understanding the clinical presentation, risk factors, treatment options and results will be advantageous for physicians in the management of patients in the future. The aim of the present study was to review and analyze the clinical manifestations, drug implications, treatment and outcome of patients with SJS and/or TEN who had been hospitalized in a tertiary care center. All hospitalized patients with SJS and/or TEN during a 5‑year period were retrospectively reviewed. The clinical severity was graded according to the score of toxic epidermal necrolysis (SCORTEN) scale. Clinical symptoms, diagnosis, possible precipitating factors, management and outcome data were collected for analysis. A total of 43 patients (mean age, 49.5 years) were hospitalized and classified into the SJS group (55.8%), SJS/TEN overlap group (20.9%) and TEN group (23.3%). The majority of the patients (90.7%) had mucocutaneous eruptions associated with oral drug administration. Allopurinol, anticonvulsants and antibiotics were the most common causative agents for the mucocutaneous eruption. Twenty‑eight patients (65.1%) were treated with corticosteroids. The mortality rate was 6.9%. Comparison between the survival group and the non‑survival group revealed that patient age >70 years (P=0.014) and body surface area involvement >20% (P<0.01) were the significant factors associated with mortality. The use of systemic steroids was higher in the survival group in comparison with the non‑survival group (65.1 vs. 0%, respectively; P=0.014). The mucocutaneous eruptions in SJS and TEN are mostly caused by medication. With early recognition and treatment, the mortality rate in this study was lower than that in previous reports. Patient age and the area of mucocutaneous involvement were significant factors associated with mortality.

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1	Mockenhaupt M: The current understanding of Stevens-Johnson syndrome and toxic epidermal necrolysis. Expert Rev Clin Immunol. 7:803–813. 2011. View Article : Google Scholar : PubMed/NCBI
2	Gerull R, Nelle M and Schaible T: Toxic epidermal necrolysis and Stevens-Johnson syndrome: A review. Crit Care Med. 39:1521–1532. 2011. View Article : Google Scholar : PubMed/NCBI
3	Mays SR, Bogle MA and Bodey GP: Cutaneous fungal infections in the oncology patient: Recognition and management. Am J Clin Dermatol. 7:31–43. 2006. View Article : Google Scholar : PubMed/NCBI
4	Bodey GP: Dermatologic manifestations of infections in neutropenic patients. Infect Dis Clin North Am. 8:655–675. 1994.PubMed/NCBI
5	Fournier S, Bastuji-Garin S, Mentec H, Revuz J and Roujeau JC: Toxic epidermal necrolysis associated with Mycoplasma pneumoniae infection. Eur J Clin Microbiol Infect Dis. 14:558–559. 1995. View Article : Google Scholar : PubMed/NCBI
6	Tanaka A, Nakano M, Tani M, Kira M, Katayama I, Nakagawa J, et al: Adult case of Stevens-Johnson syndrome possibly induced by Chlamydophila pneumoniae infection with severe involvement of bronchial epithelium resulting in constructive respiratory disorder. J Dermatol. 40:492–494. 2013. View Article : Google Scholar : PubMed/NCBI
7	Khalaf D, Toema B, Dabbour N and Jehani F: Toxic epidermal necrolysis associated with severe cytomegalovirus infection in a patient on regular hemodialysis. Mediterr J Hematol Infect Dis. 3:e20110042011. View Article : Google Scholar : PubMed/NCBI
8	Kannenberg SM, Jordaan HF, Koegelenberg CF, Von Groote-Bidlingmaier F and Visser WI: Toxic epidermal necrolysis and Stevens-Johnson syndrome in South Africa: A 3-year prospective study. QJM. 105:839–846. 2012. View Article : Google Scholar : PubMed/NCBI
9	Pavlos R, Mallal S, Ostrov D, Pompeu Y and Phillips E: Fever, rash and systemic symptoms: Understanding the role of virus and HLA in severe cutaneous drug allergy. J Allergy Clin Immunol Pract. 2:21–33. 2014. View Article : Google Scholar : PubMed/NCBI
10	Badia M, Trujillano J, Gascó E, Casanova JM, Alvarez M and León M: Skin lesions in the ICU. Intensive Care Med. 25:1271–1276. 1999. View Article : Google Scholar : PubMed/NCBI
11	Neff P, Meuli-Simmen C, Kempf W, Gaspert T, Meyer VE and Künzi W: Lyell syndrome revisited: Analysis of 18 cases of severe bullous skin disease in a burns unit. Br J Plast Surg. 58:73–80. 2005. View Article : Google Scholar : PubMed/NCBI
12	Rzany B, Mockenhaupt M, Baur S, Schröder W, Stocker U, Mueller J, et al: Epidemiology of erythema exsudativum multiforme majus, Stevens-Johnson syndrome and toxic epidermal necrolysis in Germany (1990–1992): Structure and results of a population-based registry. J Clin Epidemiol. 49:769–773. 1996. View Article : Google Scholar : PubMed/NCBI
13	Schneck J, Fagot JP, Sekula P, Sassolas B, Roujeau JC and Mockenhaupt M: Effects of treatments on the mortality of Stevens-Johnson syndrome and toxic epidermal necrolysis: A retrospective study on patients included in the prospective EuroSCAR study. J Am Acad Dermatol. 58:33–40. 2008. View Article : Google Scholar : PubMed/NCBI
14	Bastuji-Garin S, Fouchard N, Bertocchi M, Roujeau JC, Revuz J and Wolkenstein P: SCORTEN: A severity-of-illness score for toxic epidermal necrolysis. J Invest Dermatol. 115:149–153. 2000. View Article : Google Scholar : PubMed/NCBI
15	Chung WH and Hung SI: Recent advances in the genetics and immunology of Stevens-Johnson syndrome and toxic epidermal necrosis. J Dermatol Sci. 66:190–196. 2012. View Article : Google Scholar : PubMed/NCBI
16	Perkins JR, Ayuso P, Cornejo-Garcia JA and Ranea JA: The study of severe cutaneous drug hypersensitivity reactions from a systems biology perspective. Curr Opin Allergy Clin Immunol. 14:301–306. 2014. View Article : Google Scholar : PubMed/NCBI
17	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. 128:35–44. 2008. View Article : Google Scholar : PubMed/NCBI
18	Chen P, Lin JJ, Lu CS, Ong CT, Hsieh PF, Yang CC, et al: Carbamazepine-induced toxic effects and HLA-B*1502 screening in Taiwan. N Engl J Med. 364:1126–1133. 2011. View Article : Google Scholar : PubMed/NCBI
19	Mallal S, Phillips E, Carosi G, Molina JM, Workman C, Tomazic J, et al: HLA-B*5701 screening for hypersensitivity to abacavir. N Engl J Med. 358:568–579. 2008. View Article : Google Scholar : PubMed/NCBI
20	Hershfield MS, Callaghan JT, Tassaneeyakul W, Mushiroda T, Thorn CF, Klein TE, et al: Clinical Pharmacogenetics Implementation Consortium guidelines for human leukocyte antigen-B genotype and allopurinol dosing. Clin Pharmacol Ther. 93:153–158. 2013. View Article : Google Scholar : PubMed/NCBI
21	Tassaneeyakul W, Jantararoungtong T, Chen P, Lin PY, Tiamkao S, Khunarkornsiri U, et al: Strong association between HLA-B*5801 and allopurinol-induced Stevens-Johnson syndrome and toxic epidermal necrolysis in a Thai population. Pharmacogenet Genomics. 19:704–709. 2009. View Article : Google Scholar : PubMed/NCBI
22	Mockenhaupt M: Severe drug-induced skin reactions: clinical pattern, diagnostics and therapy. J Dtsch Dermatol Ges. 7:142–160. 2009. View Article : Google Scholar : PubMed/NCBI
23	Araki Y, Sotozono C, Inatomi T, Ueta M, Yokoi N, Ueda E, et al: Successful treatment of Stevens-Johnson syndrome with steroid pulse therapy at disease onset. Am J Ophthalmol. 147:1004–1011. 2009. View Article : Google Scholar : PubMed/NCBI
24	López-Garcia JS, Rivas Jara L, Garcia-Lozano CI, Conesa E, de Juan IE and Murube del Castillo J: Ocular features and histopathologic changes during follow-up of toxic epidermal necrolysis. Ophthalmology. 118:265–271. 2011. View Article : Google Scholar : PubMed/NCBI
25	De Rojas MV, Dart JK and Saw VP: The natural history of Stevens Johnson syndrome: patterns of chronic ocular disease and the role of systemic immunosuppressive therapy. Br J Ophthalmol. 91:1048–1053. 2007. View Article : Google Scholar : PubMed/NCBI
26	Prabhasawat P, Tesavibul N, Karnchanachetanee C and Kasemson S: Efficacy of cyclosporine 0.05% eye drops in Stevens Johnson syndrome with chronic dry eye. J Ocul Pharmacol Ther. 29:372–377. 2013. View Article : Google Scholar : PubMed/NCBI
27	Hague JS, Goulding JM, Long TM and Gee BC: Respiratory involvement in toxic epidermal necrolysis portends a poor prognosis that may not be reflected in SCORTEN. Br J Dermatol. 157:1294–1296. 2007. View Article : Google Scholar : PubMed/NCBI
28	Thong BY: Stevens-Johnson syndrome/toxic epidermal necrolysis: An Asia-pacific perspective. Asia Pac Allergy. 3:215–223. 2013. View Article : Google Scholar : PubMed/NCBI
29	von Wild T, Stollwerck PL, Namdar T, Stang FH, Mailänder P and Siemers F: Are multimorbidities underestimated in scoring systems of Stevens-Johnson syndrome and toxic epidermal necrolysis like in SCORTEN. Eplasty. 12:e352012.PubMed/NCBI
30	Lee HY, Dunant A, Sekula P, Mockenhaupt M, Wolkenstein P, Valeyrie-Allanore L, et al: The role of prior corticosteroid use on the clinical course of Stevens-Johnson syndrome and toxic epidermal necrolysis: a case-control analysis of patients selected from the multinational EuroSCAR and RegiSCAR studies. Br J Dermatol. 167:555–562. 2012. View Article : Google Scholar : PubMed/NCBI