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

• Published in: Anais brasileiros de dermatología Vol. 94; no. 1; pp. 17 - 23
• Main Authors: Wambier, Carlos Gustavo, Hoekstra, Thaís Angélica, Wambier, Sarah Perillo de Farias, Filho, Roberto Bueno, Vilar, Fernando Crivelenti, Paschoal, Renato Soriani, Roselino, Ana Maria, Frade, Marco Andrey Cipriani, Foss, Norma Tiraboschi
• Format: Journal Article
• Language: English
• Published: Spain Elsevier España, S.L.U 01.01.2019; Sociedade Brasileira de Dermatologia
• Subjects: Acquired immunodeficiency syndrome; Acquired Immunodeficiency Syndrome - epidemiology; Acquired Immunodeficiency Syndrome - pathology; Adolescent; Adult; Aged; Brazil - epidemiology; Child; Child, Preschool; DERMATOLOGY; Epidermal necrolysis; Epidermal necrolysis, toxic; Female; Hospitalization; Humans; Infant; Investigation; Length of Stay; Lethality; Male; Middle Aged; Poisson Distribution; Prognosis; Retrospective Studies; Risk Factors; ROC Curve; Scales; Severity of Illness Index; Statistics, Nonparametric; Stevens-Johnson syndrome; Stevens-Johnson Syndrome - epidemiology; Stevens-Johnson Syndrome - pathology; Tertiary Care Centers; Time Factors; toxic; Young Adult; Brazil; toxic; Stevens-Johnson syndrome; Epidermal necrolysis; Acquired immunodeficiency syndrome; Hospitalization; Lethality; Scales; Length of stay; Epidermal necrolysis, toxic
• ISSN: 0365-0596; 1806-4841; 1806-4841
• DOI: 10.1590/abd1806-4841.20196864

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.