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

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

doi:10.1001/jamadermatol.2023.1347

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

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

Research output: Contribution to journal › Review article › peer-review

3 Scopus citations

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.

Original language	English (US)
Pages (from-to)	772-777
Number of pages	6
Journal	JAMA Dermatology
Volume	159
Issue number	7
DOIs	https://doi.org/10.1001/jamadermatol.2023.1347
State	Published - Jul 19 2023

ASJC Scopus subject areas

Dermatology

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10.1001/jamadermatol.2023.1347

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Waters, M., Dobry, A., Le, S. T., Shinkai, K., Beachkofsky, T. M., Davis, M. D. P., Dominguez, A. R., Kroshinsky, D., Markova, A., Micheletti, R. G., Mostaghimi, A., Pasieka, H. B., Rosenbach, M., Seminario-Vidal, L., Trinidad, J., Albrecht, J., Altman, E. M., Arakaki, R., Ardern-Jones, M., ... Kaffenberger, B. H. (2023). Development of a Skin-Directed Scoring System for Stevens-Johnson Syndrome and Epidermal Necrolysis: A Delphi Consensus Exercise. JAMA Dermatology, 159(7), 772-777. https://doi.org/10.1001/jamadermatol.2023.1347

Waters, M, Dobry, A, Le, ST, Shinkai, K, Beachkofsky, TM, Davis, MDP, Dominguez, AR, Kroshinsky, D, Markova, A, Micheletti, RG, Mostaghimi, A, Pasieka, HB, Rosenbach, M, Seminario-Vidal, L, Trinidad, J, Albrecht, J, Altman, EM, Arakaki, R, Ardern-Jones, M, Bridges, AG, Cardones, AR, Chadha, AA, Chen, JK, Chen, ST, Cheng, K, Daveluy, S, Deniro, KL, Harp, J, Keller, JJ, King, B, Korman, AM, Lowenstein, EJ, Luxenberg, E, Mancuso, JB, Mauskar, MM, Milam, P, Motaparthi, K, Nelson, CA, Nguyen, CV, Nutan, F, Ortega-Loayza, AG, Patel, T, Rahnama-Moghadam, S, Rekhtman, S, Rojek, NW, Sarihan, M, Shaigany, S, Sharma, TR, Shearer, SM, Shields, BE, Strowd, LC, Tartar, DM, Thomas, C, Wanat, KA, Walls, AC, Zaba, LC, Ziemer, CM, Maverakis, E & Kaffenberger, BH 2023, 'Development of a Skin-Directed Scoring System for Stevens-Johnson Syndrome and Epidermal Necrolysis: A Delphi Consensus Exercise', JAMA Dermatology, vol. 159, no. 7, pp. 772-777. https://doi.org/10.1001/jamadermatol.2023.1347

@article{10080546aa1c4353aa449c805a3c4970,

title = "Development of a Skin-Directed Scoring System for Stevens-Johnson Syndrome and Epidermal Necrolysis: A Delphi Consensus Exercise",

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.",

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

year = "2023",

month = jul,

day = "19",

doi = "10.1001/jamadermatol.2023.1347",

language = "English (US)",

volume = "159",

pages = "772--777",

journal = "JAMA Dermatology",

issn = "2168-6068",

publisher = "American Medical Association",

number = "7",

}

TY - JOUR

T1 - Development of a Skin-Directed Scoring System for Stevens-Johnson Syndrome and Epidermal Necrolysis

T2 - A Delphi Consensus Exercise

AU - Waters, Margo

AU - Dobry, Allison

AU - Le, Stephanie T.

AU - Shinkai, Kanade

AU - Beachkofsky, Thomas M.

AU - Davis, Mark D.P.

AU - Dominguez, Arturo R.

AU - Kroshinsky, Daniela

AU - Markova, Alina

AU - Micheletti, Robert G.

AU - Mostaghimi, Arash

AU - Pasieka, Helena B.

AU - Rosenbach, Misha

AU - Seminario-Vidal, Lucia

AU - Trinidad, John

AU - Albrecht, Joerg

AU - Altman, Emily M.

AU - Arakaki, Ryan

AU - Ardern-Jones, Michael

AU - Bridges, Alina G.

AU - Cardones, Adela R.

AU - Chadha, Angad A.

AU - Chen, Jennifer K.

AU - Chen, Steven T.

AU - Cheng, Kyle

AU - Daveluy, Steven

AU - Deniro, Katherine L.

AU - Harp, Joanna

AU - Keller, Jesse J.

AU - King, Brett

AU - Korman, Abraham M.

AU - Lowenstein, Eve J.

AU - Luxenberg, Erin

AU - Mancuso, Jennifer Brescoll

AU - Mauskar, Melissa M.

AU - Milam, Philip

AU - Motaparthi, Kiran

AU - Nelson, Caroline A.

AU - Nguyen, Cuong V.

AU - Nutan, Fnu

AU - Ortega-Loayza, Alex G.

AU - Patel, Tejesh

AU - Rahnama-Moghadam, Sahand

AU - Rekhtman, Sergey

AU - Rojek, Nathan W.

AU - Sarihan, Mansi

AU - Shaigany, Sheila

AU - Sharma, Timmie R.

AU - Shearer, Sabrina M.

AU - Shields, Bridget E.

AU - Strowd, Lindsay C.

AU - Tartar, Danielle M.

AU - Thomas, Cristina

AU - Wanat, Karolyn A.

AU - Walls, Andrew C.

AU - Zaba, Lisa C.

AU - Ziemer, Carolyn M.

AU - Maverakis, Emanual

AU - Kaffenberger, Benjamin H.

PY - 2023/7/19

Y1 - 2023/7/19

N2 - 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.

AB - 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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SN - 2168-6068

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JO - JAMA Dermatology

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

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