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Epidermal necrolysis: SCORTEN performance in AIDS and non-AIDS patients

BACKGROUND: Stevens-Johnson syndrome and toxic epidermal necrolysis are life-threatening blistering drug reactions with high incidence of ocular sequela. The term ‘Epidermal Necrolysis’ has been recently used to better describe the full spectrum of the disease that includes Stevens-Johnson syndrome...

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Autores principales: Wambier, Carlos Gustavo, Hoekstra, Thaís Angélica, Wambier, Sarah Perillo de Farias, Bueno Filho, Roberto, Vilar, Fernando Crivelenti, Paschoal, Renato Soriani, Roselino, Ana Maria, Frade, Marco Andrey Cipriani, Foss, Norma Tiraboschi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Sociedade Brasileira de Dermatologia 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6360969/
https://www.ncbi.nlm.nih.gov/pubmed/30726458
http://dx.doi.org/10.1590/abd1806-4841.20196864
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author Wambier, Carlos Gustavo
Hoekstra, Thaís Angélica
Wambier, Sarah Perillo de Farias
Bueno Filho, Roberto
Vilar, Fernando Crivelenti
Paschoal, Renato Soriani
Roselino, Ana Maria
Frade, Marco Andrey Cipriani
Foss, Norma Tiraboschi
author_facet Wambier, Carlos Gustavo
Hoekstra, Thaís Angélica
Wambier, Sarah Perillo de Farias
Bueno Filho, Roberto
Vilar, Fernando Crivelenti
Paschoal, Renato Soriani
Roselino, Ana Maria
Frade, Marco Andrey Cipriani
Foss, Norma Tiraboschi
author_sort Wambier, Carlos Gustavo
collection PubMed
description BACKGROUND: Stevens-Johnson syndrome and toxic epidermal necrolysis are life-threatening blistering drug reactions with high incidence of ocular sequela. The term ‘Epidermal Necrolysis’ has been recently used to better describe the full spectrum of the disease that includes Stevens-Johnson syndrome and toxic epidermal necrolysis at opposite ends, which differ by the extent of body surface area with epidermal detachment. SCORTEN is a mortality prognosis score for ‘Epidermal Necrolysis’ cases that still needed validation in acquired immunodeficiency syndrome. OBJECTIVE: To evaluate the SCORTEN performance in acquired immunodeficiency syndrome, and the differences in outcomes between acquired immunodeficiency syndrome and non- acquired immunodeficiency syndrome cohorts. METHODS: Retrospective cohort study of AIDS and non-AIDS ‘Epidermal Necrolysis’ cases admitted to a Brazilian reference center from 1990-2014. RESULTS: Five deaths (16.7%) occurred as a consequence of EN in 30 AIDS patients, and seven (17.9%) in 39 non-AIDS patients, relative risk (RR) .92 (p=1.0). SCORTEN showed great performance, with an Area Under the Receiver Operating Curve (AUC) (ROC) of 0.90 with a 95% confidence interval ranging from .81 to .99. The performance of SCORTEN was better among non- AIDS patients than AIDS patients: AUC non- acquired immunodeficiency syndrome =0.99 (CI 05% 0.96-1.00), AUC acquired immunodeficiency syndrome = 0.74 (CI 95% 0.53-0.95), p=.02. STUDY LIMITATIONS: Heterogeneity of cases, wide variation of systemic corticosteroid doses when used. CONCLUSION: SCORTEN is valid for the Brazilian population, including among those patients with acquired immunodeficiency syndrome, and, as such, its use is recommended for aiding treatment choice in this subgroup of patients.
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spelling pubmed-63609692019-02-11 Epidermal necrolysis: SCORTEN performance in AIDS and non-AIDS patients Wambier, Carlos Gustavo Hoekstra, Thaís Angélica Wambier, Sarah Perillo de Farias Bueno Filho, Roberto Vilar, Fernando Crivelenti Paschoal, Renato Soriani Roselino, Ana Maria Frade, Marco Andrey Cipriani Foss, Norma Tiraboschi An Bras Dermatol Investigation BACKGROUND: Stevens-Johnson syndrome and toxic epidermal necrolysis are life-threatening blistering drug reactions with high incidence of ocular sequela. The term ‘Epidermal Necrolysis’ has been recently used to better describe the full spectrum of the disease that includes Stevens-Johnson syndrome and toxic epidermal necrolysis at opposite ends, which differ by the extent of body surface area with epidermal detachment. SCORTEN is a mortality prognosis score for ‘Epidermal Necrolysis’ cases that still needed validation in acquired immunodeficiency syndrome. OBJECTIVE: To evaluate the SCORTEN performance in acquired immunodeficiency syndrome, and the differences in outcomes between acquired immunodeficiency syndrome and non- acquired immunodeficiency syndrome cohorts. METHODS: Retrospective cohort study of AIDS and non-AIDS ‘Epidermal Necrolysis’ cases admitted to a Brazilian reference center from 1990-2014. RESULTS: Five deaths (16.7%) occurred as a consequence of EN in 30 AIDS patients, and seven (17.9%) in 39 non-AIDS patients, relative risk (RR) .92 (p=1.0). SCORTEN showed great performance, with an Area Under the Receiver Operating Curve (AUC) (ROC) of 0.90 with a 95% confidence interval ranging from .81 to .99. The performance of SCORTEN was better among non- AIDS patients than AIDS patients: AUC non- acquired immunodeficiency syndrome =0.99 (CI 05% 0.96-1.00), AUC acquired immunodeficiency syndrome = 0.74 (CI 95% 0.53-0.95), p=.02. STUDY LIMITATIONS: Heterogeneity of cases, wide variation of systemic corticosteroid doses when used. CONCLUSION: SCORTEN is valid for the Brazilian population, including among those patients with acquired immunodeficiency syndrome, and, as such, its use is recommended for aiding treatment choice in this subgroup of patients. Sociedade Brasileira de Dermatologia 2019 /pmc/articles/PMC6360969/ /pubmed/30726458 http://dx.doi.org/10.1590/abd1806-4841.20196864 Text en http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial No Derivative License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium provided the original work is properly cited and the work is not changed in any way.
spellingShingle Investigation
Wambier, Carlos Gustavo
Hoekstra, Thaís Angélica
Wambier, Sarah Perillo de Farias
Bueno Filho, Roberto
Vilar, Fernando Crivelenti
Paschoal, Renato Soriani
Roselino, Ana Maria
Frade, Marco Andrey Cipriani
Foss, Norma Tiraboschi
Epidermal necrolysis: SCORTEN performance in AIDS and non-AIDS patients
title Epidermal necrolysis: SCORTEN performance in AIDS and non-AIDS patients
title_full Epidermal necrolysis: SCORTEN performance in AIDS and non-AIDS patients
title_fullStr Epidermal necrolysis: SCORTEN performance in AIDS and non-AIDS patients
title_full_unstemmed Epidermal necrolysis: SCORTEN performance in AIDS and non-AIDS patients
title_short Epidermal necrolysis: SCORTEN performance in AIDS and non-AIDS patients
title_sort epidermal necrolysis: scorten performance in aids and non-aids patients
topic Investigation
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6360969/
https://www.ncbi.nlm.nih.gov/pubmed/30726458
http://dx.doi.org/10.1590/abd1806-4841.20196864
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