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Development of a skin-directed scoring system for Stevens-Johnson syndrome and epidermal necrolysis: a Delphi consensus exercise

Development of a skin-directed scoring system for Stevens-Johnson syndrome and epidermal necrolysis: a Delphi consensus exercise
Development of a skin-directed scoring system for Stevens-Johnson syndrome and epidermal necrolysis: a Delphi consensus exercise
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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Waters, Margo, Dobry, Allison, Le, Stephanie T., Shinkai, Kanade, Beachkofsky, Thomas M., Davis, Mark D. P., Dominguez, Arturo R., Kroshinsky, Daniela, Markova, Alina, Micheletti, Robert G., Mostaghimi, Arash, Pasieka, Helena B., Rosenbach, Misha, Seminario-vidal, Lucia, Trinidad, John, Albrecht, Joerg, Altman, Emily M., Arakaki, Ryan, Ardern-jones, Michael, Bridges, Alina G., Cardones, Adela R., Chadha, Angad A., Chen, Jennifer K., Chen, Steven T., Cheng, Kyle, Daveluy, Steven, Deniro, Katherine L., Harp, Joanna, Keller, Jesse J., King, Brett, Korman, Abraham M., Lowenstein, Eve J., Luxenberg, Erin, Mancuso, Jennifer Brescoll, Mauskar, Melissa M., Milam, Philip, Motaparthi, Kiran, Nelson, Caroline A., Nguyen, Cuong V., Nutan, Fnu, Ortega-loayza, Alex G., Patel, Tejesh, Rahnama-moghadam, Sahand, Rekhtman, Sergey, Rojek, Nathan W., Sarihan, Mansi, Shaigany, Sheila, Sharma, Timmie R., Shearer, Sabrina M., Shields, Bridget E., Strowd, Lindsay C., Tartar, Danielle M., Thomas, Cristina, Wanat, Karolyn A., Walls, Andrew C., Zaba, Lisa C., Ziemer, Carolyn M., Maverakis, Emanual and Kaffenberger, Benjamin H. (2023) Development of a skin-directed scoring system for Stevens-Johnson syndrome and epidermal necrolysis: a Delphi consensus exercise. Archives of Dermatology, 159 (7), 772-777. (doi:10.1001/jamadermatol.2023.1347).

Record type: Article

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.

Text
SJS Delphi Manuscript for Experts - Accepted Manuscript
Available under License University of Southampton Accepted Manuscript Licence.
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e-pub ahead of print date: 31 May 2023
Published date: 1 July 2023
Additional Information: Funding Information: Funding: The Dermatology Foundation financially supported Dr Kaffenberger. Publisher Copyright: © 2023 American Medical Association. All rights reserved.
Related URLs:
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Identifiers

Local EPrints ID: 481172
URI: http://eprints.soton.ac.uk/id/eprint/481172
DOI: doi:10.1001/jamadermatol.2023.1347
ISSN: 2168-6068
PURE UUID: 2321bb1f-abb5-4c8a-abed-1bcea35dc5bf
ORCID for Michael Ardern-jones: ORCID iD orcid.org/0000-0003-1466-2016
ORCID for Andrew C. Walls: ORCID iD orcid.org/0000-0003-4803-4595

Catalogue record

Date deposited: 17 Aug 2023 16:47
Last modified: 18 Mar 2024 03:07

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Contributors

Author: Margo Waters
Author: Allison Dobry
Author: Stephanie T. Le
Author: Kanade Shinkai
Author: Thomas M. Beachkofsky
Author: Mark D. P. Davis
Author: Arturo R. Dominguez
Author: Daniela Kroshinsky
Author: Alina Markova
Author: Robert G. Micheletti
Author: Arash Mostaghimi
Author: Helena B. Pasieka
Author: Misha Rosenbach
Author: Lucia Seminario-vidal
Author: John Trinidad
Author: Joerg Albrecht
Author: Emily M. Altman
Author: Ryan Arakaki
Author: Michael Ardern-jones ORCID iD
Author: Alina G. Bridges
Author: Adela R. Cardones
Author: Angad A. Chadha
Author: Jennifer K. Chen
Author: Steven T. Chen
Author: Kyle Cheng
Author: Steven Daveluy
Author: Katherine L. Deniro
Author: Joanna Harp
Author: Jesse J. Keller
Author: Brett King
Author: Abraham M. Korman
Author: Eve J. Lowenstein
Author: Erin Luxenberg
Author: Jennifer Brescoll Mancuso
Author: Melissa M. Mauskar
Author: Philip Milam
Author: Kiran Motaparthi
Author: Caroline A. Nelson
Author: Cuong V. Nguyen
Author: Fnu Nutan
Author: Alex G. Ortega-loayza
Author: Tejesh Patel
Author: Sahand Rahnama-moghadam
Author: Sergey Rekhtman
Author: Nathan W. Rojek
Author: Mansi Sarihan
Author: Sheila Shaigany
Author: Timmie R. Sharma
Author: Sabrina M. Shearer
Author: Bridget E. Shields
Author: Lindsay C. Strowd
Author: Danielle M. Tartar
Author: Cristina Thomas
Author: Karolyn A. Wanat
Author: Andrew C. Walls ORCID iD
Author: Lisa C. Zaba
Author: Carolyn M. Ziemer
Author: Emanual Maverakis
Author: Benjamin H. Kaffenberger

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