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      Management of Stevens-Johnson Syndrome-Toxic Epidermal Necrolysis: Looking Beyond Guidelines!

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          Abstract

          Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe cutaneous adverse reactions, which are mainly caused by drugs; and these are usually associated with high degree of morbidity and mortality. Recently, two detailed guidelines were published on the management of SJS/TEN, Indian guidelines and UK guidelines. Still, there is no consensus on the management of SJS/TEN. In this article, our aim is to conceptualize the management aspect of SJS/TEN considering Indian setup. Early discontinuation of all medicines, supportive measures (hydration, electrolytes, and care of denuded skin), corticosteroids and cyclosporine has been found to be useful. Oral provocation test is reserved for patients, who undergo complete remission and this is to be done after hospitalization, under strict vigilance. As there is no consensus, the treatment should be individualized on case to case basis.

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          Toxic epidermal necrolysis and Stevens-Johnson syndrome

          Thomas Harr, Lars E. French (2010)
          Toxic epidermal necrolysis (TEN) and Stevens Johnson Syndrome (SJS) are severe adverse cutaneous drug reactions that predominantly involve the skin and mucous membranes. Both are rare, with TEN and SJS affecting approximately 1or 2/1,000,000 annually, and are considered medical emergencies as they are potentially fatal. They are characterized by mucocutaneous tenderness and typically hemorrhagic erosions, erythema and more or less severe epidermal detachment presenting as blisters and areas of denuded skin. Currently, TEN and SJS are considered to be two ends of a spectrum of severe epidermolytic adverse cutaneous drug reactions, differing only by their extent of skin detachment. Drugs are assumed or identified as the main cause of SJS/TEN in most cases, but Mycoplasma pneumoniae and Herpes simplex virus infections are well documented causes alongside rare cases in which the aetiology remains unknown. Several drugs are at "high" risk of inducing TEN/SJS including: Allopurinol, Trimethoprim-sulfamethoxazole and other sulfonamide-antibiotics, aminopenicillins, cephalosporins, quinolones, carbamazepine, phenytoin, phenobarbital and NSAID's of the oxicam-type. Genetic susceptibility to SJS and TEN is likely as exemplified by the strong association observed in Han Chinese between a genetic marker, the human leukocyte antigen HLA-B*1502, and SJS induced by carbamazepine. Diagnosis relies mainly on clinical signs together with the histological analysis of a skin biopsy showing typical full-thickness epidermal necrolysis due to extensive keratinocyte apoptosis. Differential diagnosis includes linear IgA dermatosis and paraneoplastic pemphigus, pemphigus vulgaris and bullous pemphigoid, acute generalized exanthematous pustulosis (AGEP), disseminated fixed bullous drug eruption and staphyloccocal scalded skin syndrome (SSSS). Due to the high risk of mortality, management of patients with SJS/TEN requires rapid diagnosis, evaluation of the prognosis using SCORTEN, identification and interruption of the culprit drug, specialized supportive care ideally in an intensive care unit, and consideration of immunomodulating agents such as high-dose intravenous immunoglobulin therapy. SJS and TEN are severe and life-threatening. The average reported mortality rate of SJS is 1-5%, and of TEN is 25-35%; it can be even higher in elderly patients and those with a large surface area of epidermal detachment. More than 50% of patients surviving TEN suffer from long-term sequelae of the disease.
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            Inhibition of toxic epidermal necrolysis by blockade of CD95 with human intravenous immunoglobulin.

            P Wehrli, J-H Saurat, J Tschopp … (1998)
            Toxic epidermal necrolysis (TEN, Lyell's syndrome) is a severe adverse drug reaction in which keratinocytes die and large sections of epidermis separate from the dermis. Keratinocytes normally express the death receptor Fas (CD95); those from TEN patients were found to express lytically active Fas ligand (FasL). Antibodies present in pooled human intravenous immunoglobulins (IVIG) blocked Fas-mediated keratinocyte death in vitro. In a pilot study, 10 consecutive individuals with clinically and histologically confirmed TEN were treated with IVIG; disease progression was rapidly reversed and the outcome was favorable in all cases. Thus, Fas-FasL interactions are directly involved in the epidermal necrolysis of TEN, and IVIG may be an effective treatment.
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              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.

              Jürgen Schneck, Jean-Paul Fagot, Peggy Sekula … (2008)
              No treatment modality has been established as standard for patients with Stevens-Johnson syndrome and toxic epidermal necrolysis. We sought to evaluate the effect of treatment on mortality in a large cohort of patients with Stevens-Johnson syndrome or toxic epidermal necrolysis. Data on therapy were retrospectively collected from patients in France and Germany enrolled in EuroSCAR, a case-control study of risk factors. Neither intravenous immunoglobulins nor corticosteroids showed any significant effect on mortality in comparison with supportive care only. Compared with supportive care, odds ratios for death were 1.4 (95% confidence interval: 0.6-4.3) for intravenous immunoglobulins in France and 1.5 (0.5-4.4) in Germany, and 0.4 (0.1-1.7) for corticosteroids in France and 0.3 (0.1-1.1) in Germany. Such an observational study with retrospective data collection has obvious limitations, including heterogeneity between the countries, supportive care, treatment doses, and durations. We found no sufficient evidence of a benefit for any specific treatment. The trend for a beneficial effect of corticosteroids deserves further exploration.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Indian J Dermatol
                Journal ID (iso-abbrev): Indian J Dermatol
                Journal ID (publisher-id): IJD
                Title: Indian Journal of Dermatology
                Publisher: Medknow Publications & Media Pvt Ltd (India )
                ISSN (Print): 0019-5154
                ISSN (Electronic): 1998-3611
                Publication date (Print): Mar-Apr 2018
                Volume: 63
                Issue: 2
                Pages: 117-124
                Affiliations
                [1] From the Department of Dermatology, Grant Medical College, Mumbai, Maharashtra, India
                [1 ] Department of Dermatology, KPC Medical College, Kolkata, West Bengal, India
                [2 ] Department of Dermatology, Calcutta National Medical College, Kolkata, West Bengal, India
                Author notes
                Address for correspondence: Dr. Rajesh Kumar, Department of Dermatology, Grant Medical College, Mumbai, Maharashtra, India. E-mail: rkderm@ 123456gmail.com
                Article
                Publisher ID: IJD-63-117
                DOI: 10.4103/ijd.IJD_583_17
                PMC ID: 5903040
                PubMed ID: 29692452
                SO-VID: c8681fae-7738-48f2-8768-e1b07fb5052d
                Copyright © Copyright: © Indian Journal of Dermatology
                License:

                This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.

                History
                Date received : December 2017
                Date accepted : February 2018
                Categories
                Subject: IJD Symposium

                ScienceOpen disciplines: Dermatology
                Keywords: management,stevens-johnson syndrome,toxic epidermal necrolysis
                Data availability:
                ScienceOpen disciplines: Dermatology
                Keywords: management, stevens-johnson syndrome, toxic epidermal necrolysis

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