Post-acute phase and sequelae management of epidermal necrolysis: an international, multidisciplinary DELPHI-based consensus

S. Ingen-Housz-Oro; V. Schmidt; M. M. Ameri; R. Abe; A. Brassard; A. Mostaghimi; A. S. Paller; A. Romano; B. Didona; B. H. Kaffenberger; B. Ben Said; B. Y.H. Thong; B. Ramsay; E. Brezinova; B. Milpied; C. G. Mortz; C. Y. Chu; C. Sotozono; J. Gueudry; D. G. Fortune; S. M. Dridi; D. Tartar; G. Do-Pham; E. Gabison; E. J. Phillips; F. Lewis; C. Salavastru; B. Horvath; J. Dart; J. Setterfield; J. Newman; J. T. Schulz; A. Delcampe; K. Brockow; L. Seminario-Vidal; L. Jörg; M. P. Watson; M. Gonçalo; M. Lucas; M. Torres; M. H. Noe; N. Hama; N. H. Shear; P. O’Reilly; P. Wolkenstein; P. Romanelli; R. P. Dodiuk-Gad; S. Ahmad; S. T. Le; S. Walsh

doi:10.1186/s13023-023-02631-7

Post-acute phase and sequelae management of epidermal necrolysis: an international, multidisciplinary DELPHI-based consensus

S. Ingen-Housz-Oro^*, V. Schmidt, M. M. Ameri^*, R. Abe, A. Brassard, A. Mostaghimi, A. S. Paller, A. Romano, B. Didona, B. H. Kaffenberger, B. Ben Said, B. Y.H. Thong, B. Ramsay, E. Brezinova, B. Milpied, C. G. Mortz, C. Y. Chu, C. Sotozono, J. Gueudry, D. G. FortuneS. M. Dridi, D. Tartar, G. Do-Pham, E. Gabison, E. J. Phillips, F. Lewis, C. Salavastru, B. Horvath, J. Dart, J. Setterfield, J. Newman, J. T. Schulz, A. Delcampe, K. Brockow, L. Seminario-Vidal, L. Jörg, M. P. Watson, M. Gonçalo, M. Lucas, M. Torres, M. H. Noe, N. Hama, N. H. Shear, P. O’Reilly, P. Wolkenstein, P. Romanelli, R. P. Dodiuk-Gad, S. Ahmad, S. T. Le, S. Walsh

^*Corresponding author for this work

Translational Immunology Groningen (TRIGR)

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Abstract

Background: Long-term sequelae are frequent and often disabling after epidermal necrolysis (Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN)). However, consensus on the modalities of management of these sequelae is lacking. Objectives: We conducted an international multicentric DELPHI exercise to establish a multidisciplinary expert consensus to standardize recommendations regarding management of SJS/TEN sequelae. Methods: Participants were sent a survey via the online tool “Survey Monkey” consisting of 54 statements organized into 8 topics: general recommendations, professionals involved, skin, oral mucosa and teeth, eyes, genital area, mental health, and allergy workup. Participants evaluated the level of appropriateness of each statement on a scale of 1 (extremely inappropriate) to 9 (extremely appropriate). Results were analyzed according to the RAND/UCLA Appropriateness Method. Results: Fifty-two healthcare professionals participated. After the first round, a consensus was obtained for 100% of 54 initially proposed statements (disagreement index < 1). Among them, 50 statements were agreed upon as ‘appropriate’; four statements were considered ‘uncertain’, and ultimately finally discarded. Conclusions: Our DELPHI-based expert consensus should help guide physicians in conducting a prolonged multidisciplinary follow-up of sequelae in SJS-TEN.

Original language	English
Article number	33
Number of pages	10
Journal	Orphanet journal of rare diseases
Volume	18
Issue number	1
DOIs	https://doi.org/10.1186/s13023-023-02631-7
Publication status	Published - 22-Feb-2023

Keywords

Consensus
Delphi
Epidermal necrolysis
Quality of life
Sequelae
Stevens-Johnson syndrome
Toxic epidermal necrolysis

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Ingen-Housz-Oro, S., Schmidt, V., Ameri, M. M., Abe, R., Brassard, A., Mostaghimi, A., Paller, A. S., Romano, A., Didona, B., Kaffenberger, B. H., Ben Said, B., Thong, B. Y. H., Ramsay, B., Brezinova, E., Milpied, B., Mortz, C. G., Chu, C. Y., Sotozono, C., Gueudry, J., ... Walsh, S. (2023). Post-acute phase and sequelae management of epidermal necrolysis: an international, multidisciplinary DELPHI-based consensus. Orphanet journal of rare diseases, 18(1), Article 33. https://doi.org/10.1186/s13023-023-02631-7

@article{b4a375177aaa483f8d0b0497b5fcfc74,

title = "Post-acute phase and sequelae management of epidermal necrolysis: an international, multidisciplinary DELPHI-based consensus",

abstract = "Background: Long-term sequelae are frequent and often disabling after epidermal necrolysis (Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN)). However, consensus on the modalities of management of these sequelae is lacking. Objectives: We conducted an international multicentric DELPHI exercise to establish a multidisciplinary expert consensus to standardize recommendations regarding management of SJS/TEN sequelae. Methods: Participants were sent a survey via the online tool “Survey Monkey” consisting of 54 statements organized into 8 topics: general recommendations, professionals involved, skin, oral mucosa and teeth, eyes, genital area, mental health, and allergy workup. Participants evaluated the level of appropriateness of each statement on a scale of 1 (extremely inappropriate) to 9 (extremely appropriate). Results were analyzed according to the RAND/UCLA Appropriateness Method. Results: Fifty-two healthcare professionals participated. After the first round, a consensus was obtained for 100% of 54 initially proposed statements (disagreement index < 1). Among them, 50 statements were agreed upon as {\textquoteleft}appropriate{\textquoteright}; four statements were considered {\textquoteleft}uncertain{\textquoteright}, and ultimately finally discarded. Conclusions: Our DELPHI-based expert consensus should help guide physicians in conducting a prolonged multidisciplinary follow-up of sequelae in SJS-TEN.",

keywords = "Consensus, Delphi, Epidermal necrolysis, Quality of life, Sequelae, Stevens-Johnson syndrome, Toxic epidermal necrolysis",

author = "S. Ingen-Housz-Oro and V. Schmidt and Ameri, {M. M.} and R. Abe and A. Brassard and A. Mostaghimi and Paller, {A. S.} and A. Romano and B. Didona and Kaffenberger, {B. H.} and {Ben Said}, B. and Thong, {B. Y.H.} and B. Ramsay and E. Brezinova and B. Milpied and Mortz, {C. G.} and Chu, {C. Y.} and C. Sotozono and J. Gueudry and Fortune, {D. G.} and Dridi, {S. M.} and D. Tartar and G. Do-Pham and E. Gabison and Phillips, {E. J.} and F. Lewis and C. Salavastru and B. Horvath and J. Dart and J. Setterfield and J. Newman and Schulz, {J. T.} and A. Delcampe and K. Brockow and L. Seminario-Vidal and L. J{\"o}rg and Watson, {M. P.} and M. Gon{\c c}alo and M. Lucas and M. Torres and Noe, {M. H.} and N. Hama and Shear, {N. H.} and P. O{\textquoteright}Reilly and P. Wolkenstein and P. Romanelli and Dodiuk-Gad, {R. P.} and S. Ahmad and Le, {S. T.} and S. Walsh",

note = "Funding Information: The work was funded by ERN skin. Publisher Copyright: {\textcopyright} 2023, The Author(s).",

year = "2023",

month = feb,

day = "22",

doi = "10.1186/s13023-023-02631-7",

language = "English",

volume = "18",

journal = "Orphanet journal of rare diseases",

issn = "1750-1172",

publisher = "BMC",

number = "1",

}

Ingen-Housz-Oro, S, Schmidt, V, Ameri, MM, Abe, R, Brassard, A, Mostaghimi, A, Paller, AS, Romano, A, Didona, B, Kaffenberger, BH, Ben Said, B, Thong, BYH, Ramsay, B, Brezinova, E, Milpied, B, Mortz, CG, Chu, CY, Sotozono, C, Gueudry, J, Fortune, DG, Dridi, SM, Tartar, D, Do-Pham, G, Gabison, E, Phillips, EJ, Lewis, F, Salavastru, C, Horvath, B, Dart, J, Setterfield, J, Newman, J, Schulz, JT, Delcampe, A, Brockow, K, Seminario-Vidal, L, Jörg, L, Watson, MP, Gonçalo, M, Lucas, M, Torres, M, Noe, MH, Hama, N, Shear, NH, O’Reilly, P, Wolkenstein, P, Romanelli, P, Dodiuk-Gad, RP, Ahmad, S, Le, ST & Walsh, S 2023, 'Post-acute phase and sequelae management of epidermal necrolysis: an international, multidisciplinary DELPHI-based consensus', Orphanet journal of rare diseases, vol. 18, no. 1, 33. https://doi.org/10.1186/s13023-023-02631-7

TY - JOUR

T1 - Post-acute phase and sequelae management of epidermal necrolysis

T2 - an international, multidisciplinary DELPHI-based consensus

AU - Ingen-Housz-Oro, S.

AU - Schmidt, V.

AU - Ameri, M. M.

AU - Abe, R.

AU - Brassard, A.

AU - Mostaghimi, A.

AU - Paller, A. S.

AU - Romano, A.

AU - Didona, B.

AU - Kaffenberger, B. H.

AU - Ben Said, B.

AU - Thong, B. Y.H.

AU - Ramsay, B.

AU - Brezinova, E.

AU - Milpied, B.

AU - Mortz, C. G.

AU - Chu, C. Y.

AU - Sotozono, C.

AU - Gueudry, J.

AU - Fortune, D. G.

AU - Dridi, S. M.

AU - Tartar, D.

AU - Do-Pham, G.

AU - Gabison, E.

AU - Phillips, E. J.

AU - Lewis, F.

AU - Salavastru, C.

AU - Horvath, B.

AU - Dart, J.

AU - Setterfield, J.

AU - Newman, J.

AU - Schulz, J. T.

AU - Delcampe, A.

AU - Brockow, K.

AU - Seminario-Vidal, L.

AU - Jörg, L.

AU - Watson, M. P.

AU - Gonçalo, M.

AU - Lucas, M.

AU - Torres, M.

AU - Noe, M. H.

AU - Hama, N.

AU - Shear, N. H.

AU - O’Reilly, P.

AU - Wolkenstein, P.

AU - Romanelli, P.

AU - Dodiuk-Gad, R. P.

AU - Ahmad, S.

AU - Le, S. T.

AU - Walsh, S.

PY - 2023/2/22

Y1 - 2023/2/22

N2 - Background: Long-term sequelae are frequent and often disabling after epidermal necrolysis (Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN)). However, consensus on the modalities of management of these sequelae is lacking. Objectives: We conducted an international multicentric DELPHI exercise to establish a multidisciplinary expert consensus to standardize recommendations regarding management of SJS/TEN sequelae. Methods: Participants were sent a survey via the online tool “Survey Monkey” consisting of 54 statements organized into 8 topics: general recommendations, professionals involved, skin, oral mucosa and teeth, eyes, genital area, mental health, and allergy workup. Participants evaluated the level of appropriateness of each statement on a scale of 1 (extremely inappropriate) to 9 (extremely appropriate). Results were analyzed according to the RAND/UCLA Appropriateness Method. Results: Fifty-two healthcare professionals participated. After the first round, a consensus was obtained for 100% of 54 initially proposed statements (disagreement index < 1). Among them, 50 statements were agreed upon as ‘appropriate’; four statements were considered ‘uncertain’, and ultimately finally discarded. Conclusions: Our DELPHI-based expert consensus should help guide physicians in conducting a prolonged multidisciplinary follow-up of sequelae in SJS-TEN.

AB - Background: Long-term sequelae are frequent and often disabling after epidermal necrolysis (Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN)). However, consensus on the modalities of management of these sequelae is lacking. Objectives: We conducted an international multicentric DELPHI exercise to establish a multidisciplinary expert consensus to standardize recommendations regarding management of SJS/TEN sequelae. Methods: Participants were sent a survey via the online tool “Survey Monkey” consisting of 54 statements organized into 8 topics: general recommendations, professionals involved, skin, oral mucosa and teeth, eyes, genital area, mental health, and allergy workup. Participants evaluated the level of appropriateness of each statement on a scale of 1 (extremely inappropriate) to 9 (extremely appropriate). Results were analyzed according to the RAND/UCLA Appropriateness Method. Results: Fifty-two healthcare professionals participated. After the first round, a consensus was obtained for 100% of 54 initially proposed statements (disagreement index < 1). Among them, 50 statements were agreed upon as ‘appropriate’; four statements were considered ‘uncertain’, and ultimately finally discarded. Conclusions: Our DELPHI-based expert consensus should help guide physicians in conducting a prolonged multidisciplinary follow-up of sequelae in SJS-TEN.

KW - Consensus

KW - Delphi

KW - Epidermal necrolysis

KW - Quality of life

KW - Sequelae

KW - Stevens-Johnson syndrome

KW - Toxic epidermal necrolysis

U2 - 10.1186/s13023-023-02631-7

DO - 10.1186/s13023-023-02631-7

M3 - Article

C2 - 36814255

AN - SCOPUS:85148722700

SN - 1750-1172

VL - 18

JO - Orphanet journal of rare diseases

JF - Orphanet journal of rare diseases

IS - 1

M1 - 33

ER -

Post-acute phase and sequelae management of epidermal necrolysis: an international, multidisciplinary DELPHI-based consensus

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Keywords

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