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Dermatology

Development of a Skin-Directed Scoring System for Stevens-Johnson Syndrome and Epidermal NecrolysisA Delphi Consensus Exercise

To identify the key insights or developments described in this article

1 Credit CME

JAMA Dermatology

Published Online: May 31, 2023

Consensus Statement

Article Information and Disclosures

Margo Waters, BS¹;

Allison Dobry, MD²;

Stephanie T. Le, MD³;

Kanade Shinkai, MD, PhD²;

Thomas M. Beachkofsky, MD⁴;

Mark D. P. Davis, MD⁵;

Arturo R. Dominguez, MD⁶;

Daniela Kroshinsky, MD, MPH⁷;

Alina Markova, MD⁸;

Robert G. Micheletti, MD⁹;

Arash Mostaghimi, MD, MPA, MPH¹⁰;

Helena B. Pasieka, MD, MS^11,12;

Misha Rosenbach, MD⁹;

Lucia Seminario-Vidal, MD, PhD¹³;

John Trinidad, MD, MPH⁷;

Joerg Albrecht, MD, PhD¹⁴;

Emily M. Altman, MD¹⁵;

Ryan Arakaki, MD²;

Michael Ardern-Jones, DPhil¹⁶;

Alina G. Bridges, DO¹⁷;

Adela R. Cardones, MD, MHSc¹⁸;

Angad A. Chadha, MD¹⁹;

Jennifer K. Chen, MD²⁰;

Steven T. Chen, MD, MPH, MHPEd⁷;

Kyle Cheng, MD²¹;

Steven Daveluy, MD²²;

Katherine L. DeNiro, MD²³;

Joanna Harp, MD²⁴;

Jesse J. Keller, MD, MCR²⁵;

Brett King, MD, PhD²⁶;

Abraham M. Korman, MD²⁷;

Eve J. Lowenstein, MD, PhD²⁸;

Erin Luxenberg, MD²⁹;

Jennifer Brescoll Mancuso, MD³⁰;

Melissa M. Mauskar, MD⁶;

Philip Milam, MD³¹;

Kiran Motaparthi, MD³²;

Caroline A. Nelson, MD²⁶;

Cuong V. Nguyen, MD³³;

Fnu Nutan, MD³⁴;

Alex G. Ortega-Loayza, MD, MCR²⁵;

Tejesh Patel, MD³⁵;

Sahand Rahnama-Moghadam, MD, MS³⁶;

Sergey Rekhtman, MD, PharmD, MPH¹⁷;

Nathan W. Rojek, MD³⁷;

Mansi Sarihan, MD³⁸;

Sheila Shaigany, MD¹⁷;

Timmie R. Sharma, MD³⁹;

Sabrina M. Shearer, MD¹⁸;

Bridget E. Shields, MD⁴⁰;

Lindsay C. Strowd, MD⁴¹;

Danielle M. Tartar, MD, PhD³;

Cristina Thomas, MD⁶;

Karolyn A. Wanat, MD⁴²;

Andrew C. Walls, MD¹⁰;

Lisa C. Zaba, MD, PhD²⁰;

Carolyn M. Ziemer, MD, MPH⁴³;

Emanual Maverakis, MD³;

Benjamin H. Kaffenberger, MD, MS²⁷

Author Affiliations

¹The Ohio State University College of Medicine, Columbus
²Department of Dermatology, University of California, San Francisco
³Department of Dermatology, University of California, Davis
⁴Department of Dermatology, James A. Haley Veterans Hospital, Tampa, Florida
⁵Department of Dermatology, Mayo Clinic, Rochester, Minnesota
⁶Departments of Internal Medicine and Dermatology, University of Texas Southwestern Medical Center, Dallas
⁷Department of Dermatology, Massachusetts General Hospital, Boston
⁸Department of Dermatology, Memorial Sloan Kettering Cancer Center, New York, New York
⁹Department of Dermatology and Internal Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia
¹⁰Department of Dermatology, Brigham and Women’s Hospital and Harvard Medical School, Boston, Massachusetts
¹¹Departments of Dermatology, Uniformed Services University, Bethesda, Maryland
¹²The Burn Center, MedStar Washington Hospital Center, Washington, DC
¹³Department of Dermatology, University of South Florida, Tampa
¹⁴Department of Medicine, Division of Dermatology, Cook County Health, Chicago, Illinois
¹⁵Department of Dermatology, University of New Mexico Health Sciences Center, Albuquerque
¹⁶Department of Dermatology, University of Southampton, Southampton, UK
¹⁷Departments of Dermatology and Anatomic Pathology, Donald and Barbara Zucker School for Medicine at Hofstra/Northwell, New Hyde Park, New York
¹⁸Department of Dermatology, Duke University Medical Center, Durham, North Carolina
¹⁹Section of Dermatology, University of Chicago, Chicago, Illinois
²⁰Department of Dermatology, Stanford University School of Medicine, Redwood City, California
²¹Division of Dermatology, Department of Medicine, David Geffen School of Medicine at University of California, Los Angeles
²²Department of Dermatology, Wayne State University School of Medicine, Detroit, Michigan
²³Division of Dermatology, University of Washington, Seattle
²⁴Department of Dermatology, Weill Cornell Medicine, New York, New York
²⁵Department of Dermatology, Oregon Health and Science University, Portland
²⁶Department of Dermatology, Yale University School of Medicine, New Haven, Connecticut
²⁷Department of Dermatology, The Ohio State University Wexner Medical Center, Columbus
²⁸Department of Dermatology, SUNY Downstate Medical Center and Kings County Medical Center, Oceanside, New York
²⁹Department of Dermatology, Hennepin Healthcare, Minneapolis, Minnesota
³⁰Department of Dermatology, University of Michigan, Ann Arbor
³¹Department of Dermatology, Vanderbilt University Medical Center, Nashville, Tennessee
³²Department of Dermatology, University of Florida College of Medicine, Gainesville
³³Department of Dermatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois
³⁴Department of Dermatology, Virginia Commonwealth University Medical Center, Richmond
³⁵Department of Dermatology, University of Tennessee Health Science Center, Memphis
³⁶Department of Dermatology, Indiana University, Indianapolis
³⁷Department of Dermatology, University of California, Irvine
³⁸Department of Dermatology, Valleywise Health−Creighton University, University of Arizona, Mayo Clinic, Phoenix, Arizona
³⁹Department of Dermatology, University Hospitals Cleveland Medical Center, Cleveland, Ohio
⁴⁰Department of Dermatology, University of Wisconsin School of Medicine and Public Health, Madison
⁴¹Department of Dermatology, Wake Forest School of Medicine, Winston-Salem, North Carolina
⁴²Department of Dermatology, Medical College of Wisconsin, Milwaukee
⁴³Department of Dermatology, University of North Carolina Chapel Hill, Chapel Hill

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Key Points

Question Is there a need for an EN skin-directed scoring system, consistent EN terminology, a better understanding of orphologic progression, and/or identification of the most-affected sites?

Findings This Delphi consensus exercise determined that there is a need for a skin-directed scoring system for EN with consensus achieved regarding terminology, morphologic traits, and most-affected locations of involvement.

Meaning The findings of this consensus exercise may be used as a framework for developing a skin-directed EN grading tool with the goal of precisely monitoring disease severity and changes during hospitalization.

Abstract

Importance Scoring systems for Stevens-Johnson syndrome and epidermal necrolysis (EN) only estimate patient prognosis and are weighted toward comorbidities and systemic features; morphologic terminology for EN lesions is inconsistent.

Objectives To establish consensus among expert dermatologists on EN terminology, morphologic progression, and most-affected sites, and to build a framework for developing a skin-directed scoring system for EN.

Evidence Review A Delphi consensus using the RAND/UCLA appropriateness criteria was initiated with a core group from the Society of Dermatology Hospitalists to establish agreement on the optimal design for an EN cutaneous scoring instrument, terminology, morphologic traits, and sites of involvement.

Findings In round 1, the 54 participating dermatology hospitalists reached consensus on all 49 statements (30 appropriate, 3 inappropriate, 16 uncertain). In round 2, they agreed on another 15 statements (8 appropriate, 7 uncertain). There was consistent agreement on the need for a skin-specific instrument; on the most-often affected skin sites (head and neck, chest, upper back, ocular mucosa, oral mucosa); and that blanching erythema, dusky erythema, targetoid erythema, vesicles/bullae, desquamation, and erosions comprise the morphologic traits of EN and can be consistently differentiated.

Conclusions and Relevance This consensus exercise confirmed the need for an EN skin-directed scoring system, nomenclature, and differentiation of specific morphologic traits, and identified the sites most affected. It also established a baseline consensus for a standardized EN instrument with consistent terminology.

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Article Information

Accepted for Publication: April 2, 2023.

Published Online: May 31, 2023. doi:10.1001/jamadermatol.2023.1347

Correction: This article was corrected on July 19, 2023, to correct the spelling of an author’s surname.

Corresponding Author: Benjamin H. Kaffenberger, MD, MS, Department of Dermatology, The Ohio State University Wexner Medical Center, 1328 Dublin Rd, Ste 100, Columbus, OH 43215 (benjamin.kaffenberger@osumc.edu).

Author Contributions: Dr Kaffenberger had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. Ms Waters and Drs Dobry and Le should be considered co−first authors.

Concept and design: Waters, Dobry, Le, Shinkai, Trinidad, Korman, Lowenstein, Rahnama-Moghadam, Maverakis, Kaffenberger.

Acquisition, analysis, or interpretation of data: Waters, Dobry, Le, Beachkofsky, Davis, Dominguez, Kroshinsky, Markova, Micheletti, Mostaghimi, Pasieka, Rosenbach, Seminario-Vidal, Trinidad, Albrecht, Altman, Ardern-Jones, Bridges, Cardones, Chadha, J. Chen, Cheng, Daveluy, DeNiro, Harp, Keller, Korman, Luxenberg, Mancuso, Milam, Motaparthi, Nelson, Nguyen, Nutan, Ortega-Loayza, Patel, Rahnama-Moghadam, Rojek, Sarihan, Sharma, Shearer, Shields, Strowd, Tartar, Wanat, Ziemer, Maverakis, Kaffenberger, Arakaki, S. Chen, King, Mauskar, Rekhtman, Shaigany, Thomas, Walls, Zaba.

Drafting of the manuscript: Waters, Dobry, Le, Markova, Seminario-Vidal, Trinidad, Altman, Bridges, Korman, Maverakis.

Critical revision of the manuscript for important intellectual content: Le, Shinkai, Beachkofsky, Davis, Dominguez, Kroshinsky, Markova, Micheletti, Mostaghimi, Pasieka, Rosenbach, Trinidad, Albrecht, Altman, Ardern-Jones, Bridges, Cardones, Chadha, J. Chen, Cheng, Daveluy, DeNiro, Harp, Keller, Korman, Lowenstein, Luxenberg, Mancuso, Milam, Motaparthi, Nelson, Nguyen, Nutan, Ortega-Loayza, Patel, Rahnama-Moghadam, Rojek, Sarihan, Sharma, Shearer, Shields, Strowd, Tartar, Wanat, Ziemer, Maverakis, Kaffenberger, Arakaki, S. Chen, King, Mauskar, Rekhtman, Shaigany, Thomas, Walls, Zaba.

Statistical analysis: Waters, Le, Markova, Bridges, Rahnama-Moghadam, Maverakis.

Administrative, technical, or material support: Le, Beachkofsky, Pasieka, Trinidad, Bridges, Korman, Milam, Nelson, Ortega-Loayza, Rahnama-Moghadam, Wanat, Maverakis, Kaffenberger, Mauskar, Shaigany.

Supervision: Le, Kroshinsky, Markova, Mostaghimi, Seminario-Vidal, Trinidad, DeNiro, Harp, Mancuso, Rahnama-Moghadam, Rojek, Sharma, Wanat, Ziemer, Maverakis, Kaffenberger.

Other−participation in the Delphi process: Albrecht, Nutan, Rosenbach, Tartar.

Other−technical contribution: Ardern-Jones.

Other−participation in survey as clinical expert: Pasieka.

Other−data contribution: Dominguez.

Other−Delphi consensus: Altman.

Conflict of Interest Disclosures: Dr Beachkofsky reported grants from the US Air Force, the Defense Health Agency, and the Veterans Administration outside the submitted work. Dr Markova reported grants from the US National Institutes of Health/National Cancer Institute (No. P30-CA008748) during the conduct of the study; personal fees from ADC Therapeutics, Blueprint Medicines, Alira Health, Protagonist Therapeutics, OnQuality Pharmaceuticals, and Janssen, and a patent and royalties for UpToDate, all outside the submitted work. Dr Micheletti reported personal fees from Vertex, grants from Acelyrin, ChemoCentryx, Amgen, Pfizer, and Cabaletta Bio outside the submitted work. Dr Mostaghimi reported personal fees from Pfizer, Hims and Hers, Digital Diagnostics, Concert, Lilly, AbbVie, Equillium, ACOM, Boehringer Ingelheim, and LEO Pharma outside the submitted work; and personal fees from the artwork in Figure 1 of this study. Dr Rosenbach reported personal fees from Processa Pharma, Merck, Novartis, and CSL Behring; grants from Processa, AbbVie, and Johnson & Johnson, outside the submitted work. Dr Seminario-Vidal reported personal fees and grants from Eli Lilly and Novartis; grants from Celgene, Kyowa Kirin, Soligenix, Helsinn, Eisai, Boehringer Ingelheim, Amgen, AnaptysBio, Regeneron, Blueprint, AbbVie, Bristol Myers Squibb, Glenmark, and Innate Pharma outside the submitted work. Dr Daveluy reported personal fees from AbbVie, UCB, and Pfizer outside the submitted work. Dr S. Chen reported personal fees from Pfizer, Novartis, Scholar Rock, and VisualDx outside the submitted work. No other disclosures were reported.

Funding: The Dermatology Foundation financially supported Dr Kaffenberger.

Role of Funder: The Dermatology Foundation had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; nor the decision to submit the manuscript for publication.

Disclaimer: The opinions and assertions expressed herein are those of the author(s) and do not reflect the official policy or position of the Uniformed Services University of the Health Sciences or the US Department of Defense. Dr Kanade Shinkai is the Chief Editor of JAMA Dermatology, Dr Robert G. Micheletti is editor of “Images in Dermatology” for JAMA Dermatology, and Dr Misha Rosenbach serves on the Editorial Board of JAMA Dermatology; however, they were not involved in any of the decisions regarding review of the manuscript or its acceptance.

AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
1.00 credit toward the CME [and Self-Assessment requirements] of the American Board of Surgery’s Continuous Certification program

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