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

Dokumentationszentrum schwerer Hautreaktionen, University Medical Center Freiburg, Freiburg, Germany.
Journal of the American Academy of Dermatology (Impact Factor: 5). 02/2008; 58(1):33-40. DOI: 10.1016/j.jaad.2007.08.039
Source: PubMed

ABSTRACT 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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    • "intravenous immunoglobulin therapy (IVIG), thalidomide, and TNF-í µí»¼ antagonist. Traditionally systemic corticosteroids were advocated until early 1990s, although no benefit has been demonstrated in case-controlled studies [6]. A retrospective single center study proposes that short-term dexamethasone therapy, given at an early stage of the disease, may contribute to a reduced mortality rate [7]. "
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    ABSTRACT: Background. Stevens-Johnson syndrome (SJS) and/or toxic epidermal necrolysis (TEN) are uncommon and life-threatening drug reaction associated with a high morbidity and mortality. Objective. We studied SJS and/or TEN by conducting a retrospective analysis of 87 patients treated during a 10-year period. Methods. We conducted a retrospective review of the records of all patients with a diagnosis of SJS and/or TEN based on clinical features and histological confirmation of SJS and/or TEN was not available at the Department of Medicine, Vajira hospital, Bangkok, Thailand. The data were collected from two groups from 2003 to 2007 and 2008 to 2012. Results. A total of 87 cases of SJS and/or TEN were found, comprising 44 males and 43 females whose mean age was 46.5 years. The average length of stay was 17 days. Antibiotics, anticonvulsants, and allopurinol were the major culprit drugs in both groups. The mean SCORTEN on admission was 2.1 in first the group while 1.7 in second the group. From 2008 to 2012, thirty-nine patients (76.5%) were treated with corticosteroids while only eight patients (22.2%) were treated between 2003 and 2007. The mortality rate declined from 25% from the first group to 13.7% in the second group. Complications between first and second groups had no significant differences. Conclusions. Short-term corticosteroids may contribute to a reduced mortality rate in SJS and/or TEN without increasing secondary infection. Further well-designed studies are required to compare the effect of corticosteroids treatment for SJS and/or TEN.
    Dermatology Research and Practice 06/2014; 2014:237821. DOI:10.1155/2014/237821
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    • "Trent Viard Prins Campione Al-Mutairi Shortt Tan Stella Rajaratnam Bachot Brown Schneck 2003 1998 2003 2003 2004 2004 2005 2007 2010 2003 2004 2008 (80) (44) (73) (76) (74) (78) (79) (82) (81) (72) (75) (69) Study design Retro Prosp Retro Prosp Prosp Retro Retro Retro Retro Prosp Retro Retro N/ N/ N/ N/ N/ N/ N/ Cont N/ N/ N/ Cont Cont Cont Cont Cont Cont Cont Cont Cont Cont Cont Patients 24 10 48 10 12 16 12 23 14 34 24 75 Detachm.(%) 44 39 45 49 58 " 65 " — — " 44 " 19 49 — Total dose IVIG (g/kg) 4 3 3 2 2-5 2.8 2 — 3.3 2 1.6 1.9 (0.7-2.3) Predicted mortality (SCORTEN/APACHE*) in % 33 — — 35 — 38 * — 35.8 36 24 28.6 25 Actual mortality in % 4 0 12 10 0 25 8 26 21 32 41.7 34 Harr and French Orphanet Journal of Rare Diseases 2010, 5:39 "
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    ABSTRACT: 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.
    Orphanet Journal of Rare Diseases 12/2010; 5(1):39. DOI:10.1186/1750-1172-5-39 · 3.96 Impact Factor
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