Khosrow S Houschyar1, Christian Tapking2,3, Mimi R Borrelli4, Behrus Puladi5, Mark Ooms5, Christoph Wallner6, Dominik Duscher7, Dominik Pförringer8, Susanne Rein9, Georg Reumuth10, Torsten Schulz11, Ina Nietzschmann11, Zeshaan N Maan4, Gerrit Grieb12, Wolfgang G Philipp-Dormston13, Ludwik K Branski2, Frank Siemers11, Marcus Lehnhardt6, Laurenz Schmitt1, Amir S Yazdi1. 1. Department of Dermatology and Allergology, University Hospital Aachen, Germany. 2. Department of Surgery, Shriners Hospitals for Children-Galveston, University of Texas Medical Branch, 815 Market Street, Galveston, TX 77550, US. 3. Department of Hand, Plastic and Reconstructive Surgery, Burn Trauma Center, BG Trauma Center Ludwigshafen, University of Heidelberg, Germany. 4. Division of Plastic and Reconstructive Surgery, Department of Surgery, Stanford School of Medicine, Stanford, CA 94305, US. 5. Department of Oral and Maxillofacial Surgery, University Hospital RWTH, Aachen. 6. Department of Plastic Surgery, BG University Hospital Bergmannsheil, Ruhr University Bochum, Bürkle-de-la-Camp Platz 1, 44789, Bochum, Germany. 7. Department of Plastic Surgery and Hand Surgery, Technical University Munich, Munich, Germany. 8. Clinic and Policlinic of Trauma Surgery, Klinikum Rechts der Isar, Technische Universität München, Germany. 9. Department of Plastic and Hand Surgery-Burn Center-Clinic St. Georg, Leipzig, Germany. 10. Department of Plastic Surgery and Hand Surgery, Evangelische Elisabeth Klinik, Luetzowstraße 26, 10785 Berlin, Germany. 11. Department of Plastic and Hand Surgery, Burn Unit, Trauma Center Bergmannstrost Halle, Halle, Germany. 12. Department of Plastic Surgery and Hand Surgery, Gemeinschaftskrankenhaus Havelhoehe, Teaching Hospital of the Charité Berlin, Kladower Damm 221, 14089 Berlin, Germany. 13. Hautzentrum Köln/Cologne Dermatology, Cologne, Germany.
Abstract
OBJECTIVE: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare and severe skin and mucosal reactions that are associated with high mortality. Despite the severity, an evidence-based treatment protocol for SJS/TEN is still lacking. METHOD: In this systematic review and meta-analysis, the PubMed database was searched using the following terms: [Stevens-Johnson syndrome] OR [toxic epidermal necrolysis] AND [therapy] OR [treatment] over a 20-year period (1999-2019) in the German and English language. All clinical studies reporting on the treatment of SJS/TEN were included, and epidemiological and diagnostic aspects of treatment were analysed. A meta-analysis was conducted on all comparative clinical studies that met the inclusion criteria. RESULTS: A total of 88 studies met the inclusion criteria, reporting outcomes in 2647 patients. Treatment was either supportive or used systemic corticosteroid, intravenous immunoglobulin, plasmapheresis, cyclosporine, thalidomide or cyclophosphamide therapy. The meta-analysis included 16 (18%) studies, reporting outcomes in 976 (37%) patients. Systemic glucocorticoids showed a survival benefit for SJS/TEN patients in all analyses compared with other forms of treatment. Cyclosporine treatment also showed promising results, despite being used in a small cohort of patients. No beneficial effects on mortality could be demonstrated for intravenous immunoglobulins. CONCLUSION: Glucocorticoids and cyclosporine may be tentatively recommended as the most promising immunomodulatory therapies for SJS/TEN, but these results should be investigated in future prospective controlled trials.
OBJECTIVE: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare and severe skin and mucosal reactions that are associated with high mortality. Despite the severity, an evidence-based treatment protocol for SJS/TEN is still lacking. METHOD: In this systematic review and meta-analysis, the PubMed database was searched using the following terms: [Stevens-Johnson syndrome] OR [toxic epidermal necrolysis] AND [therapy] OR [treatment] over a 20-year period (1999-2019) in the German and English language. All clinical studies reporting on the treatment of SJS/TEN were included, and epidemiological and diagnostic aspects of treatment were analysed. A meta-analysis was conducted on all comparative clinical studies that met the inclusion criteria. RESULTS: A total of 88 studies met the inclusion criteria, reporting outcomes in 2647 patients. Treatment was either supportive or used systemic corticosteroid, intravenous immunoglobulin, plasmapheresis, cyclosporine, thalidomide or cyclophosphamide therapy. The meta-analysis included 16 (18%) studies, reporting outcomes in 976 (37%) patients. Systemic glucocorticoids showed a survival benefit for SJS/TEN patients in all analyses compared with other forms of treatment. Cyclosporine treatment also showed promising results, despite being used in a small cohort of patients. No beneficial effects on mortality could be demonstrated for intravenous immunoglobulins. CONCLUSION: Glucocorticoids and cyclosporine may be tentatively recommended as the most promising immunomodulatory therapies for SJS/TEN, but these results should be investigated in future prospective controlled trials.