Stevens-Johnson Syndrome – clinical symptoms, etiology, pathophysiology and medical treatment - a review of the literature
DOI:
https://doi.org/10.12775/JEHS.2022.12.11.032Keywords
Stevens-Johnson Syndrome, cutaneous adverse drug reactions, acute skin drug reaction, epidermal necrolysis, life-threatening disease of skinAbstract
Stevens-Johnson syndrome (SJS) is a rare disease of the skin and mucous membrane. It develops as a result of a sudden skin reaction, which is most often triggered by the use of certain drugs. It is characterized by acute epidermal necrosis.
The syndrome can lead to many complications and death. Currently, there are no guidelines and management schematics for this disease. Treatment has changed over the years due to the discovery of the possible pathomechanism of SJS. Nevertheless, because of the rarity of the disease, there is a lack of adequate prospective randomized studies that could provide valuable information on treatment.
Objective:
The purpose of this study was to present Stevens-Johnson syndrome, including clinical features, etiology, pathomechanism, complications and treatment.
Methods:
A literature search was performed in the PubMed medical publication database using the following keywords: Stevens-Johnson syndrome, acute drug-induced skin reaction, cutaneous adverse drug reactions.
Results:
Stevens-Johnson syndrome is an example of an acute cutaneous reaction to certain medicinal substances. The pathomechanism is not fully understood. There is an association of genetics with the risk of developing symptoms of the syndrome in selected populations. Treatment formerly was based on glucocorticosteroids, but now IVIG (intravenous immune globulin) is used. Dehydration, pneumonia or sepsis may develop as a complication of the syndrome.
Conclusions:
The discovery of a genetic predisposition to develop Stevens-Johnson syndrome offers the possibility of future effective disease prevention.
It is necessary to create medical procedure schemes for the diagnosis and treatment of this disorder. The discovery of the possible pathomechanism has allowed the use of IVIG in the treatment of the syndrome. Effective and prompt diagnosis and treatment can prevent life-threatening complications and death from SJS.
References
Treat J. Stevens-Johnson syndrome and toxic epidermal necrolysis. Pediatr Ann. 2010 Oct;39(10):667-9, 672-4. doi: 10.3928/00904481-20100922-11. PMID: 20954613.
Harr T, French LE. Toxic epidermal necrolysis and Stevens-Johnson syndrome. Orphanet J Rare Dis. 2010 Dec 16;5:39.
Frantz R, Huang S, Are A, Motaparthi K. Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Review of Diagnosis and Management. Medicina (Kaunas). 2021 Aug 28;57(9):895.
Ludlam G.B., Bridges J.B.: Association of Stevens-Johnson syndrome with antibody for Mycoplasma pneumoniae. Lancet, 1964; 1 (7340): 958–959
Yang C.W., Hung S.I., Juo C.G., et al.: HLAB* 1502-bound peptides: implications for the pathogenesis of carbamazepine-induced Stevens-Johnson syndrome. J. Allergy Clin. Immunol., 2007; 120 (4): 870–877
Chung WH, Hung SI, Hong HS, Hsih MS, Yang LC, Ho HC, Wu JY, Chen YT: Medical genetics: a marker for Stevens-Johnson syndrome. Nature 2004, 428:486.
Tassaneeyakul W, Tiamkao S, Jantararoungtong T, Chen P, Lin SY, Chen WH, Konyoung P, Khunarkornsiri U, Auvichayapat N, Pavakul K, et al: Association between HLA-B*1502 and carbamazepine-induced severe cutaneous adverse drug reactions in a Thai population. Epilepsia 2010, 51:926-930
Lonjou C, Thomas L, Borot N, Ledger N, de Toma C, LeLouet H, Graf E, Schumacher M, Hovnanian A, Mockenhaupt M, Roujeau JC: A marker for Stevens-Johnson syndrome...: ethnicity matters. Pharmacogenomics J 2006, 6:265-268.
Ueta M., Tokunaga K., Sotozono C., et al.: HLA class I and II gene polymorphisms in Stevens-Johnson syndrome with ocular complications in Japanese. Mol. Vis., 2008; 14: 550–555
Viard I., Wehrli P., Bullani R., et al.: Inhibition of toxic epidermal necrolysis by blockade of CD95 with human intravenous immunoglobulin. Science, 1998; 282 (5388): 490–493
Chung W.-H., Hung S.-I., Yang J.-Y., Su S.-C., Huang S.-P., Wei C.-Y., Chin S.-W., Chiou C.-C., Chu S.-C., Ho H.-C., et al. Granulysin is a key mediator for disseminated keratinocyte death in Stevens-Johnson syndrome and Toxic Epidermal Necrolysis. Nat. Med. 2008;14:1343–1350
Su S.-C., Mockenhaupt M., Wolkenstein P., Dunant A., le Gouvello S., Chen C.-B., Chosidow O., Valeyrie-Allanore L., Bellon T., Sekula P., et al. Interleukin-15 Is Associated with Severity and Mortality in Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis. J. Investig. Dermatol. 2017;137:1065–1073
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, 136:323-327.
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, 27:26-33
Coias J.L., Abbas L.F., Cardones A.R. Management of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: A Review and Update. Curr. Dermatol. Rep. 2019;8:219–233
Creamer D., Walsh S.A., Dziewulski P., Exton L.S., Lee H.Y., Dart J.K.G., Setterfield J., Bunker C.B., Ardern-Jones M.R., Watson K.M.T., et al. UK guidelines for the management of Ste-vens-Johnson syndrome/Toxic Epidermal Necrolysis in adults 2016. J. Plast. Reconstr. Aesthetic Surg. 2016;69:e119–e153
Ng Q.X., De Deyn M.L.Z.Q., Venkatanarayanan N., Ho C.Y.X., Yeo W.-S. A meta-analysis of cyclosporine treatment for Stevens–Johnson Syndrome/Toxic Epidermal Necrolysis. J. Inflamm. Res. 2018;11:135–142
Chen Y.-T., Hsu C.-Y., Chien Y.-N., Lee W.-R., Huang Y.-C. Efficacy of cyclosporine for the treatment of Stevens-Johnson syndrome and Toxic Epidermal Necrolysis: Systemic review and meta-analysis. Dermatol. Sin. 2017;35:131–137
Chan H.L., Stern R.S., Arndt K.A., et al.: The incidence of erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis. A population-based study with particular reference to reactions caused by drugs among outpatients. Arch. Dermatol., 1990; 126 (1): 43–47
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2022 Karolina Szymkiewicz, Aleksandra Marczak, Michalina Hordejuk, Michał Hyjek
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
The periodical offers access to content in the Open Access system under the Creative Commons Attribution-NonCommercial-ShareAlike 4.0
Stats
Number of views and downloads: 945
Number of citations: 0
