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      Successful Treatment of Methampyrone-Induced Toxic Epidermal Necrolysis with Therapeutic Plasma Exchange

      case-report

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          Abstract

          The toxic epidermal necrolysis (TEN) is a severe cutaneous adverse reaction frequently caused by drug exposure. A 58-year-old male was admitted to the hospital after administration of methampyrone. He developed sloughing of the total epidermis which rapidly extended over the trunk and limbs. The presumptive diagnosis was drug-induced TEN. Despite the treatment with pulsed glucocorticoid and cyclosporine, the skin lesions extended over the entire body. Strikingly, the progression of blistering was stopped by therapeutic plasma exchange (TPE). TPE was discontinued after the signs of skin inflammation had been overcome. He recovered in 8 days of hospitalization. We present here a case of a methampyrone-induced TEN which was successfully treated with TPE.

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

          S Bastuji-Garin, N Fouchard, M. Bertocchi … (2000)
          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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            Guidelines on the Use of Therapeutic Apheresis in Clinical Practice-Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Seventh Special Issue.

            Joseph Schwartz, Anand Padmanabhan, Nicole A Aqui … (2016)
            The American Society for Apheresis (ASFA) Journal of Clinical Apheresis (JCA) Special Issue Writing Committee is charged with reviewing, updating, and categorizing indications for the evidence-based use of therapeutic apheresis in human disease. Since the 2007 JCA Special Issue (Fourth Edition), the Committee has incorporated systematic review and evidence-based approaches in the grading and categorization of apheresis indications. This Seventh Edition of the JCA Special Issue continues to maintain this methodology and rigor to make recommendations on the use of apheresis in a wide variety of diseases/conditions. The JCA Seventh Edition, like its predecessor, has consistently applied the category and grading system definitions in the fact sheets. The general layout and concept of a fact sheet that was used since the fourth edition has largely been maintained in this edition. Each fact sheet succinctly summarizes the evidence for the use of therapeutic apheresis in a specific disease entity. The Seventh Edition discusses 87 fact sheets (14 new fact sheets since the Sixth Edition) for therapeutic apheresis diseases and medical conditions, with 179 indications, which are separately graded and categorized within the listed fact sheets. Several diseases that are Category IV which have been described in detail in previous editions and do not have significant new evidence since the last publication are summarized in a separate table. The Seventh Edition of the JCA Special Issue serves as a key resource that guides the utilization of therapeutic apheresis in the treatment of human disease. J. Clin. Apheresis 31:149-162, 2016. © 2016 Wiley Periodicals, Inc.
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              Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Concise Review with a Comprehensive Summary of Therapeutic Interventions Emphasizing Supportive Measures

              Jeremy A. Schneider, Philip R. Cohen (2017)
              Introduction Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are two of the most severe dermatologic conditions occurring in the inpatient setting. There is a lack of consensus regarding appropriate management of SJS and TEN. Purpose The scientific literature pertaining to SJS and TEN (subsequently referred to as SJS/TEN) is summarized and assessed. In addition, an interventional approach for the clinician is provided. Methods PubMed was searched with the key words: corticosteroids, cyclosporine, etanercept, intravenous immunoglobulin, Stevens-Johnson syndrome, and toxic epidermal necrolysis. The papers generated by the search, and their references, were reviewed. Results Supportive care is the most universally accepted intervention for SJS/TEN. Specific guidelines differ from the care required for patients with thermal burns. Adjuvant therapies are utilized in most severe cases, but the data are thus far underwhelming and underpowered. Using systemic corticosteroids as sole therapy is not supported. A consensus regarding combined corticosteroids and intravenous immunoglobulin (IVIG) has not been reached. Data regarding IVIG, currently the standard of care for most referral centers, is conflicting. Newer studies regarding cyclosporine and tumor necrosis factor inhibitors are promising, but not powered to provide definitive evidence of efficacy. Data regarding plasmapheresis is equivocal. Thalidomide increases mortality. Conclusion Clinicians who manage SJS/TEN should seek to employ interventions with the greatest impact on their patients’ condition. While supportive care measures may seem an obvious aspect of SJS/TEN patient care, providers should understand that these interventions are imperative and that they differ from the care recommended for other critically ill or burn patients. While adjuvant therapies are frequently discussed and debated for hospitalized patients with SJS/TEN, a standardized management approach is not yet clear based on the current data. Therefore, until further data are available, decisions regarding such treatments should be made on a case-by-case basis.
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                Author and article information

                Contributors
                Santosa:
                ORCID: http://orcid.org/0000-0002-6093-5049
                Journal
                Journal ID (nlm-ta): Case Rep Med
                Journal ID (iso-abbrev): Case Rep Med
                Journal ID (publisher-id): CRIM
                Title: Case Reports in Medicine
                Publisher: Hindawi
                ISSN (Print): 1687-9627
                ISSN (Electronic): 1687-9635
                Publication date Collection: 2018
                Publication date (Electronic): 18 July 2018
                Volume: 2018
                Electronic Location Identifier: 2182604
                Affiliations
                1Division of Hematology and Medical Oncology, Department of Internal Medicine, Medical Faculty of Diponegoro University and Dr. Kariadi Hospital, Semarang, Indonesia
                2Department of Internal Medicine, Medical Faculty of Mulawarman University, A. M. Parikesit Hospital and A. W. Sjahranie Hospital, Tenggarong-Samarinda, Indonesia
                3Department of Dermatology and Venereology, Telogorejo Hospital, Semarang, Indonesia
                Author notes

                Academic Editor: Georgios D. Kotzalidis

                Author information
                http://orcid.org/0000-0002-6093-5049
                http://orcid.org/0000-0002-9777-9705
                Article
                DOI: 10.1155/2018/2182604
                PMC ID: 6079558
                PubMed ID: 30123277
                SO-VID: 73a2b3d9-81c2-4b6d-8fd5-6974ccc0add8
                Copyright © Copyright © 2018 Santosa et al.
                License:

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 15 April 2018
                Date accepted : 12 June 2018
                Funding
                Funded by: Telogerejo Hospital
                Funded by: Indonesian Red Cross
                Categories
                Subject: Case Report

                Data availability:

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