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      Bibliometric analysis of literature on toxic epidermal necrolysis and Stevens-Johnson syndrome: 1940 – 2015

      research-article
      Orphanet Journal of Rare Diseases
      BioMed Central
      Stevens-Johnsons Syndrome, Toxic epidermal necrolysis, Bibliometric analysis

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          Abstract

          Background

          Stevens Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but fatal adverse skin reactions that affect all age groups. In order to better understand literature on this topic, we conducted a bibliometric study using Scopus database to shed light on number and growth of publications, most active countries, institutions, journals and authors involved in publishing articles in this field, citation analysis, top cited articles, international collaboration, role of medications and genetic association. Bibliometric analysis will enrich the literature on these rare conditions and will provide baseline data for future comparison.

          Results

          Three thousand eight hundred fifty-six journal articles were retrieved. The h-index of retrieved documents was 95. Growth rates of publications were highest from 1966 to 1975 and from 2006 to 2015. The United States of America ( n = 640; 16.57%) was the leading country in number of publications. However, French and Japanese researchers and institutions were most active in publishing articles on SJS and TEN. International collaboration among active countries was relatively low and ranges from 32.5% for Swiss researchers and 1.47% for Spanish researchers. The most frequently mentioned medication in retrieved articles was carbamazepine ( n = 146) followed by phenytoin ( n = 114) and allopurinol ( n = 112). Mycoplasma infection was mentioned in 111 articles. Most documents on SJS and TEN were published in dermatology journals, specifically Archives of Dermatology. However, in the last decade, top cited articles appeared in dermatology and pharmacogenetic journals. Carbamazepine was frequently encountered with Han Chinese and HLA-B 1502 terms while allopurinol was frequently encountered with HLA-B 5801 and Japanese terms.

          Conclusion

          Bibliometric analysis reveals that research publications on SJS and TEN have been increasing since the l940s, with relatively low international collaboration. Documents are being published, not only in dermatology journals, but also in genetic, public health and general medicine journals. Research on SJS and TEN can be helpful to clinicians and researchers not only to document complications and fatal outcomes, but also to identify potential causative agents and potential ethnic variations to note gaps in research.

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          Most cited references64

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          SCORTEN: a severity-of-illness score for toxic epidermal necrolysis.

          The mortality of toxic epidermal necrolysis is about 30%. Our purpose was to develop and validate a specific severity-of-illness score for cases of toxic epidermal necrolysis admitted to a specialized unit and to compare it with the Simplified Acute Physiology Score and a burn scoring system. A sample of 165 patients was used to develop the toxic epidermal necrolysis-specific severity-of-illness score and evaluate the other scores, a sample of 75 for validation. Model development used logistic regression equations that were translated into probability of hospital mortality; validation used measures of calibration and discrimination. We identified seven independent risk factors for death and constituted the toxic epidermal necrolysis-specific severity-of-illness score: age above 40 y, malignancy, tachycardia above 120 per min, initial percentage of epidermal detachment above 10%, serum urea above 10 mmol per liter, serum glucose above 14 mmol per liter, and bicarbonate below 20 mmol per liter. For each toxic epidermal necrolysis-specific severity-of-illness score point the odds ratio was 3.45 (confidence interval 2.26-5.25). Probability of death was: P(death) = elogit/1 + elogit with logit = -4.448 + 1.237 (toxic epidermal nec-rolysis-specific severity-of-illness score). Calibration demonstrated excellent agreement between expected (19. 6%) and actual (20%) mortality; discrimination was also excellent with a receiver operating characteristic area of 82%. The Simplified Acute Physiology Score and the burn score were also associated with mortality. The discriminatory powers were poorer (receiver operating characteristic area: 72 and 75%) and calibration of the Simplified Acute Physiology Score indicated a poor agreement between expected (9.1%) and actual (26.7%) mortality. This study demonstrates that the risk of death of toxic epidermal necrolysis patients can be accurately predicted by the toxic epidermal necrolysis-specific severity-of-illness score. The Simplified Acute Physiology Score and burn score appear to be less adequate.
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            Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme.

            To conduct a prospective case-control study about causative factors of severe bullous erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis, we needed to define criteria for classifying the cases and standardize the collection of data so that cases could be reliably diagnosed according to this classification. Based on review of case histories and photographs of patients, a group of experts proposed a classification based on the pattern of erythema multiforme-like lesions (categorized as typical targets, raised or flat atypical targets, and purpuric macules) and on the extent of epidermal detachment. An atlas illustrating this classification that included photographs and schematic drawings was developed. We compared the evaluations of 28 cases by four nonphysicians relying on the atlas with the evaluations of the same cases by five experts not using the atlas to determine the usefulness of this atlas for classifying cases according to our nosologic schema. The following consensus classification in five categories was proposed: bullous erythema multiforme, detachment below 10% of the body surface area plus localized "typical targets" or "raised atypical targets"; Stevens-Johnson syndrome, detachment below 10% of the body surface area plus widespread erythematous or purpuric macules or flat atypical targets; overlap Stevens-Johnson syndrome-toxic epidermal necrolysis, detachment between 10% and 30% of the body surface area plus widespread purpuric macules or flat atypical targets; toxic epidermal necrolysis with spots, detachment above 30% of the body surface area plus widespread purpuric macules or flat atypical targets; and toxic epidermal necrolysis without spots, detachment above 10% of the body surface area with large epidermal sheets and without any purpuric macule or target. Using the atlas, the nonexperts showed excellent agreement with the experts. This study suggests that an illustrated atlas is a useful tool for standardizing the diagnosis of acute severe bullous disorders that are attributed to drugs or infectious agents. Whether the five categories proposed represent distinct etiopathologic entities will require further epidemiologic and laboratory investigations.
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              Inhibition of toxic epidermal necrolysis by blockade of CD95 with human intravenous immunoglobulin.

              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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                Author and article information

                Contributors
                +970-599-225-906 , waleedsweileh@yahoo.com , waleedsweileh@najah.edu
                Journal
                Orphanet J Rare Dis
                Orphanet J Rare Dis
                Orphanet Journal of Rare Diseases
                BioMed Central (London )
                1750-1172
                18 January 2017
                18 January 2017
                2017
                : 12
                : 14
                Affiliations
                ISNI 0000 0004 0631 5695, GRID grid.11942.3f, College of Medicine and Health Sciences, Department of Physiology and Pharmacology/Toxicology, , An-Najah National University, ; Nablus, Palestine
                Article
                566
                10.1186/s13023-017-0566-8
                5242056
                28100272
                f3f6fbb5-d6e1-43c0-bbbc-ee6b05c53b98
                © The Author(s). 2017

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

                History
                : 19 October 2016
                : 6 January 2017
                Categories
                Research
                Custom metadata
                © The Author(s) 2017

                Infectious disease & Microbiology
                stevens-johnsons syndrome,toxic epidermal necrolysis,bibliometric analysis

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