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1.
Hong Kong Med J ; 30(2): 102-109, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38531617

RESUMO

INTRODUCTION: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) [hereafter, SJS/TEN] are uncommon but severe mucocutaneous reactions. Although they have been described in many populations worldwide, data from Hong Kong are limited. Here, we explored the epidemiology, disease characteristics, aetiology, morbidity, and mortality of SJS/TEN in Hong Kong. METHODS: This retrospective cohort study included all hospitalised patients who had been diagnosed with SJS/TEN in Prince of Wales Hospital from 1 January 2004 to 31 December 2020. RESULTS: There were 125 cases of SJS/TEN during the 17-year study period. The annual incidence was 5.07 cases per million. The mean age at onset was 51.4 years. The mean maximal body surface area of epidermal detachment was 23%. Overall, patients in 32% of cases required burns unit or intensive care unit admission. Half of the cases involved concomitant sepsis, and 23.2% of cases resulted in multiorgan failure or disseminated intravascular coagulation. The mean length of stay was 23.9 days. The cause of SJS/TEN was attributed to a drug in 91.9% of cases, including 84.2% that involved anticonvulsants, allopurinol, antibiotics, or analgesics. In most cases, patients received treatment comprising either best supportive care alone (35.2%) or combined with intravenous immunoglobulin (43.2%). The in-hospital mortality rate was 21.6%. Major causes of death were multiorgan failure and/or fulminant sepsis (81.5%). CONCLUSION: This study showed that SJS/TEN are uncommon in Hong Kong but can cause substantial morbidity and mortality. Early recognition, prompt withdrawal of offending agents, and multidisciplinary supportive management are essential for improving clinical outcomes.


Assuntos
Síndrome de Stevens-Johnson , Humanos , Síndrome de Stevens-Johnson/epidemiologia , Síndrome de Stevens-Johnson/terapia , Síndrome de Stevens-Johnson/mortalidade , Síndrome de Stevens-Johnson/etiologia , Hong Kong/epidemiologia , Pessoa de Meia-Idade , Estudos Retrospectivos , Masculino , Feminino , Adulto , Incidência , Idoso , Tempo de Internação/estatística & dados numéricos , Alopurinol/efeitos adversos , Anticonvulsivantes/efeitos adversos , Anticonvulsivantes/uso terapêutico , Sepse/epidemiologia , Insuficiência de Múltiplos Órgãos/epidemiologia , Insuficiência de Múltiplos Órgãos/etiologia , Insuficiência de Múltiplos Órgãos/mortalidade
2.
BMC Med Inform Decis Mak ; 22(1): 273, 2022 10 19.
Artigo em Inglês | MEDLINE | ID: mdl-36261833

RESUMO

BACKGROUND: Toxic epidermal necrolysis (TEN) patients require multi-directional and multi-disciplinary treatment. In most cases, they are hospitalised at intensive care units and require multi-directional, burn-complication preventive care. Choosing the most appropriate treatment option might be troublesome even when predicting scores are used. SCORTEN is the most renowned prognostic score for TEN patients, however, there are some data indicating that the accuracy of this test may be limited. The credibility of not just the predicted mortality risk, but also componential laboratory results and clinical features subject to debate. The aim of this study was to evaluate the efficacy and credibility of SCORTEN in clinical practice, on proprietary material. METHODS: A retrospective analysis of 35 patients with diagnosed in histopathology TEN was performed. The inclusion criteria were as follows: day of submission before 5th day from the onset of the symptoms, full protocol of plasmaphereses and IVIGs according to our scheme. Our protocol includes cycle of plasmapheresis with frozen fresh plasma twice daily for the first 2 days following admission, and once daily for the subsequent 5 to 7 days. IVIGs were administered after the first two sessions of plasmapheresis, for 4 to 7 days. The dosage was calculated according to body weight, at 0.4 to 0.5 g/kg per dose. RESULTS: The sensitivity of SCORTEN for the analysed cohort was 100%, with a specificity of 24%. The estimated death was 41,9%, while the actual death rates were 12,5%. Our protocol improved the survival, OR = 26,57, RR = 6,34, p = 0,022. Decrease in mortality was caused by a combined treatment protocol we use- plasmaphereses with IVIGs. No independent risk factor was significant in death evaluation. CONCLUSION: Our data suggest that the scoring system for predicting death among TEN patients are reliable when they are high. New prognostic factors should be found to improve the evaluation of patients with low SCORTEN.


Assuntos
Síndrome de Stevens-Johnson , Humanos , Mortalidade Hospitalar , Unidades de Terapia Intensiva , Estudos Retrospectivos , Índice de Gravidade de Doença , Síndrome de Stevens-Johnson/diagnóstico , Síndrome de Stevens-Johnson/mortalidade , Síndrome de Stevens-Johnson/terapia
3.
J Wound Care ; 30(6): 492-496, 2021 Jun 02.
Artigo em Inglês | MEDLINE | ID: mdl-34121430

RESUMO

OBJECTIVE: Stevens-Johnson syndrome (SJS) and its more severe counterpart, toxic epidermal necrolysis (TEN), are skin hypersensitivity reactions defined by epidermal blistering and necrosis. The exact pathophysiology of SJS/TEN is yet to be deciphered, but a number of risk factors have been identified including adverse drug reactions. The diagnosis of SJS/TEN is made on a clinical basis, and treatment consists of supportive care and occasionally immunosuppressants, such as cyclosporin, high-dose intravenous immunoglobulins and/or corticosteroids. Mortality rates can reach 20-25% in adults but are reduced with early intervention. To identify optimal treatment regimens, to better understand the patient cohort affected, and to help identify key risk factors for mortality, we report our experience with the treatment and management of SJS/TEN patients. METHODS: A retrospective review of consecutive patients with SJS and/or TEN admitted to a single burns centre in Germany, between 2008 and 2018, was conducted. The primary outcomes of demographics, clinical course, treatment and patient-reported outcomes were recorded and compared with a control group of patients with burns without a diagnosis of SJS/TEN. RESULTS: A total of 23 patients with SJS/TEN met the inclusion criteria: 17 (74%) with TEN; four (17%) with SJS/TEN overlap; and two (9%) with SJS. Of the patients, 14 (61%) were female and nine (39%) were male. Patient age ranged from 32-78 years (mean: 52 years). A matched cohort of 23 patients with burns served as the control group. All patients received standard of care with a multidisciplinary team. Compared with the control group, SJS/TEN patients had higher mortality rates (n=6, 26% versus n=8, 35%, respectively). The average age of death was 69 years in SJS/TEN patients versus 63 years in control group patients. Age and SCORTEN scores were significant predictors of mortality. CONCLUSIONS: SJS and TEN are rare but extreme reactions of the skin and mucosa, associated with high disease mortality rates. This 10-year single-centre retrospective review contributes to the bank of information for reviews evaluating the management of SJS/TEN patients.


Assuntos
Corticosteroides/uso terapêutico , Queimaduras/terapia , Imunoglobulinas Intravenosas/uso terapêutico , Imunossupressores/uso terapêutico , Síndrome de Stevens-Johnson/tratamento farmacológico , Cicatrização , Adulto , Idoso , Unidades de Queimados , Queimaduras/mortalidade , Feminino , Alemanha/epidemiologia , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Síndrome de Stevens-Johnson/mortalidade , Resultado do Tratamento
4.
Med Clin North Am ; 105(4): 577-597, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-34059239

RESUMO

Severe cutaneous adverse reactions to medications (SCARs) include drug reaction with eosinophilia and systemic symptoms, Stevens-Johnson syndrome, toxic epidermal necrolysis, and acute generalized exanthematous pustulosis. They are all non-immunoglobulin E mediated hypersensitivity reaction patterns, distinguished from simple cutaneous drug eruptions by immunologic pathogenesis and internal organ involvement. Herein the clinical features, diagnostic workup, and management considerations are presented for each of these major SCARs.


Assuntos
Pustulose Exantematosa Aguda Generalizada/patologia , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/patologia , Eosinofilia/diagnóstico , Síndrome de Stevens-Johnson/etiologia , Síndrome de Stevens-Johnson/patologia , Pustulose Exantematosa Aguda Generalizada/diagnóstico , Pustulose Exantematosa Aguda Generalizada/etiologia , Corticosteroides/administração & dosagem , Corticosteroides/uso terapêutico , Adulto , Idoso , Diagnóstico Diferencial , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/diagnóstico , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/epidemiologia , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/terapia , Eosinofilia/induzido quimicamente , Feminino , Humanos , Hipersensibilidade/imunologia , Masculino , Pessoa de Meia-Idade , Farmacogenética , Prognóstico , Fatores de Risco , Índice de Gravidade de Doença , Síndrome de Stevens-Johnson/diagnóstico , Síndrome de Stevens-Johnson/mortalidade
5.
J Am Acad Dermatol ; 85(4): 873-877, 2021 10.
Artigo em Inglês | MEDLINE | ID: mdl-33940101

RESUMO

BACKGROUND: Age, bicarbonate, cancer, dialysis, 10% body surface area risk model (ABCD-10) has recently been proposed as an alternative to the SCORe of toxic epidermal necrolysis (SCORTEN) model for predicting in-hospital mortality in patients with Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN). In contrast to SCORTEN, ABCD-10 incorporates prior dialysis and upweights the impact of cancer. OBJECTIVE: To determine the performance of ABCD-10 compared with that of SCORTEN in mortality prediction at a large, tertiary burn center. METHODS: A retrospective analysis of 192 patients with SJS/TEN admitted to the North Carolina Jaycee Burn Center from January 1, 2009, to December 31, 2019, was conducted. Data on these patients were collected using the burn registry and a manual chart review. The performance of both the mortality prediction models was assessed using univariate logistic regression and the Hosmer-Lemeshow test. RESULTS: The overall mortality was 22% (n = 43). Nine (5%) patients had cancer, and 7 (4%) had undergone prior dialysis; neither factor was associated with mortality (P = .11 and P = .62, respectively). SCORTEN was well calibrated to predict inpatient mortality (P = .82), whereas ABCD-10 appeared to have a poorer fit (P < .001) in these patients. Both the models showed good discrimination. LIMITATIONS: Small sample size. CONCLUSION: SCORTEN was a better predictor of inpatient mortality than ABCD-10 in a North American cohort of patients treated at the tertiary burn center.


Assuntos
Síndrome de Stevens-Johnson , Unidades de Queimados , Estudos de Coortes , Humanos , Estudos Retrospectivos , Índice de Gravidade de Doença , Síndrome de Stevens-Johnson/mortalidade
6.
Indian J Dermatol Venereol Leprol ; 87(5): 628-644, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33871208

RESUMO

BACKGROUND: Limited evidence is available about effectiveness and choice of immunomodulating treatment modalities for toxic epidermal necrolysis (TEN). AIMS: To compare the effectiveness of interventions to reduce mortality in patients of toxic epidermal necrolysis through network meta-analysis. METHODS: Studies were retrieved using PubMed, Google Scholar and Cochrane Database of Systematic Reviews from inception to September 18, 2018. Only English language articles were considered. Observational and randomized controlled studies having ≥ 5 TEN patients in each intervention arm were included. Two investigators independently extracted study characteristics, intervention details and mortality data. Bayesian network meta-analysis was performed using the Markov chain Monte Carlo (MCMC) approach through the random effect model. The ranking analysis was done to provide a hierarchy of interventions. The consistency between direct and indirect evidence was assessed through node spit analysis. The primary outcome was to compare the mortality [Odds ratio OR (95% credibility interval CrI)] among all treatment modalities of TEN. RESULTS: Twenty-four studies satisfying the selection criteria were included. The network analysis showed improved survival with cyclosporine as compared to supportive care [OR- 0.19 (95% CrI: 0.05, 0.59)] and intravenous immunoglobulin [OR- 0.21 (95% CrI: 0.05, 0.76)]. The hierarchy of treatments based on "surface under the cumulative ranking curves" (SUCRA) value were cyclosporine (0.93), steroid+intravenous immunoglobulin (0.76), etanercept (0.59), steroids (0.46), intravenous immunoglobulin (0.40), supportive care (0.34) and thalidomide (0.02). No inconsistencies between direct and indirect estimates were observed for any of the treatment pairs. LIMITATIONS: Evidence is mainly based on retrospective studies. CONCLUSION: The use of cyclosporine can reduce mortality in TEN patients. Other promising immunomodulators could be steroid+intravenous immunoglobulin combination and etanercept.


Assuntos
Síndrome de Stevens-Johnson/terapia , Ciclosporina/uso terapêutico , Fármacos Dermatológicos/uso terapêutico , Etanercepte/uso terapêutico , Glucocorticoides/uso terapêutico , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Imunossupressores/uso terapêutico , Síndrome de Stevens-Johnson/mortalidade
7.
J Invest Dermatol ; 141(5): 1139-1140, 2021 05.
Artigo em Inglês | MEDLINE | ID: mdl-33888216

RESUMO

In-hospital mortality for epidermal necrolysis (EN) has been well-characterized, but less is known about the long-term complications. Marxer et al. (2020) report mortality rates of 7.4% during the initial hospitalization, 4.8% within 90 days, and 7.6% after 91 days. Compared with that of matched controls, long-term mortality was not increased, highlighting the importance of understanding the long-term sequelae of EN survivors.


Assuntos
Síndrome de Stevens-Johnson/complicações , Mortalidade Hospitalar , Hospitalização , Humanos , Qualidade de Vida , Síndrome de Stevens-Johnson/mortalidade , Síndrome de Stevens-Johnson/psicologia
11.
Int J Dermatol ; 60(1): 44-52, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-32686136

RESUMO

BACKGROUND: Toxic epidermal necrolysis (TEN) is a life-threatening severe cutaneous adverse reaction. Data on pediatric TEN is limited. METHODS: Case records of 44 children, 1 month-12 years with a diagnosis of TEN (>30% body surface area [%BSA] detachment) admitted to a tertiary pediatric intensive care unit (PICU) between 2009 and 2018 were analyzed retrospectively. The primary outcome was mortality, and secondary outcomes were organ dysfunction, length of stay (LOS), and long-term sequelae. RESULTS: Median (IQR) age was 6.5 (3.6, 8.0) years, and 25 (57%) were boys. Median (IQR) %BSA involved, SCORTEN score, and PRISM-III were 65% (45, 80); 2 (2, 3) and 13 (10, 16), respectively. Antiepileptics (n = 24, 54.6%) and antimicrobials (n = 8, 18.2%) were the most common offending agents. Twenty-four (54.5%) children had culture positive sepsis. Immunomodulatory therapy was provided in 35 (79.5%) and conservative management in nine (20.5%) children. Intravenous immunoglobulin (IVIG) was given in 22 (50%), steroids in three (6.8%), and both IVIG and steroids in 10 (22.7%) children. Respiratory failure (n = 14, 31.8%) was the commonest organ failure. Mortality was 15.9% (n = 7), and median (IQR) PICU-LOS in survivors was 8 (4, 11.75) days. There was no association between IVIG, steroids, or conservative management with mortality or LOS. Ocular sequelae (n = 20, 54.1%) were the most common long-term complication followed by skin (18, 40.1%). CONCLUSION: Immunomodulation with IVIG or steroids was not associated with any mortality benefit as compared to conservative management alone. Further research is required to determine the most effective treatment in pediatric TEN.


Assuntos
Cuidados Críticos , Síndrome de Stevens-Johnson/terapia , Anti-Inflamatórios/uso terapêutico , Criança , Pré-Escolar , Tratamento Conservador , Dexametasona/uso terapêutico , Oftalmopatias/etiologia , Feminino , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Imunomodulação , Lactente , Unidades de Terapia Intensiva Pediátrica , Tempo de Internação , Masculino , Metilprednisolona/uso terapêutico , Insuficiência Respiratória/etiologia , Estudos Retrospectivos , Sepse/microbiologia , Índice de Gravidade de Doença , Esteroides/uso terapêutico , Síndrome de Stevens-Johnson/complicações , Síndrome de Stevens-Johnson/mortalidade , Taxa de Sobrevida , Fatores de Tempo , Resultado do Tratamento
12.
J Am Acad Dermatol ; 84(2): 390-397, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32898587

RESUMO

BACKGROUND: Various systemic immunomodulating therapies have been used to treat toxic epidermal necrolysis (TEN), but their efficacy remains unclear. OBJECTIVE: To perform a systematic review and network meta-analysis (NMA) evaluating the effects of systemic immunomodulating therapies on mortality for Stevens-Johnson syndrome (SJS)/TEN overlap and TEN. METHODS: A literature search was performed in online databases (from inception to October 31, 2019). Outcomes were mortality rates and Score of Toxic Epidermal Necrolysis (SCORTEN)-based standardized mortality ratio (SMR). A frequentist random-effects model was adopted. RESULTS: Sixty-seven studies involving 2079 patients were included. An NMA of 10 treatments showed that none was superior to supportive care in reducing mortality rates and that thalidomide was associated with a significantly higher mortality rate (odds ratio, 11.67; 95% confidence interval [CI], 1.42-95.96). For SMR, an NMA of 11 treatment arms showed that corticosteroids and intravenous immunoglobulin combination therapy was the only treatment with significant survival benefits (SMR, 0.53; 95% CI, 0.31-0.93). LIMITATIONS: Heterogeneity and a paucity of eligible randomized controlled trials. CONCLUSIONS: Combination therapy with corticosteroids and IVIg may reduce mortality risks in patients with SJS/TEN overlap and TEN. Cyclosporine and etanercept are promising therapies, but more studies are required to provide clearer evidence.


Assuntos
Glucocorticoides/uso terapêutico , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Síndrome de Stevens-Johnson/terapia , Quimioterapia Combinada/métodos , Humanos , Metanálise em Rede , Ensaios Clínicos Controlados Aleatórios como Assunto , Síndrome de Stevens-Johnson/mortalidade , Talidomida/uso terapêutico , Resultado do Tratamento
13.
J Am Acad Dermatol ; 84(3): 644-653, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32561372

RESUMO

BACKGROUND: Complications involving internal organs are usually present in Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). However, pancreatic complications are rarely reported and studied. OBJECTIVE: To summarize clinical characteristics of SJS/TEN-associated acute pancreatic injuries and to investigate underlying inflammatory mechanisms. METHODS: Clinical records of 124 inpatients with SJS/TEN were reviewed. Serum levels of tumor necrosis factor α, interleukin (IL) 6, IL-18, IL-15, IL-12p70, and soluble CD56 were determined in 18 healthy donors and 17 patients with SJS/TEN, including 3 with acute pancreatic injuries. RESULTS: Acute pancreatic injury was diagnosed in 7.3% of patients (9/124) in the SJS/TEN cohort. Elevation of serum transaminase level and hypoalbuminemia occurred more frequently in patients with acute pancreatic injuries compared with those without pancreatic symptoms (P = .004 and <.001, respectively). Although acute pancreatic injury did not alter mortality rate of SJS/TEN, it was associated with longer hospitalization stays (P = .008). Within the serum cytokines whose levels were elevated in SJS/TEN, only IL-18 was found to be selectively increased in patients with acute pancreatic injuries compared with those without them (P = .03). LIMITATIONS: Cohort was small. CONCLUSION: Acute pancreatic injury is a gastrointestinal complication of SJS/TEN in which hepatotoxicity is more likely to occur. Overexpression of IL-18 might be involved in this unique entity.


Assuntos
Interleucina-18/sangue , Pancreatite/imunologia , Síndrome de Stevens-Johnson/complicações , Adolescente , Adulto , Idoso , Antígeno CD56/sangue , Antígeno CD56/imunologia , Criança , Feminino , Humanos , Interleucina-12/sangue , Interleucina-12/imunologia , Interleucina-15/sangue , Interleucina-15/imunologia , Interleucina-18/imunologia , Interleucina-6/sangue , Interleucina-6/imunologia , Masculino , Pessoa de Meia-Idade , Pancreatite/sangue , Estudos Retrospectivos , Síndrome de Stevens-Johnson/sangue , Síndrome de Stevens-Johnson/imunologia , Síndrome de Stevens-Johnson/mortalidade , Fator de Necrose Tumoral alfa/sangue , Fator de Necrose Tumoral alfa/imunologia , Adulto Jovem
14.
J Am Acad Dermatol ; 84(6): 1782-1791, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32828861

RESUMO

BACKGROUND: Patients with Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) have high mortality rates. Disseminated intravascular coagulation has been reported in SJS/TEN patients. The influence of this lethal complication in patients with SJS/TEN is not well known. OBJECTIVE: This study aimed to investigate the risk and outcomes of disseminated intravascular coagulation in patients with SJS/TEN. METHODS: We analyzed the disseminated intravascular coagulation profiles of patients receiving a diagnosis of SJS/TEN between 2010 and 2019. RESULTS: We analyzed 150 patients with SJS/TEN (75 with SJS, 22 with overlapping SJS/TEN, and 53 with TEN) and their complete disseminated intravascular coagulation profiles. Disseminated intravascular coagulation was diagnosed in 32 patients (21.3%), primarily those with TEN. It was significantly associated with systemic complications, including gastrointestinal bleeding, respiratory failure, renal failure, liver failure, infection, and bacteremia. Additionally, SJS/TEN patients with disseminated intravascular coagulation had elevated procalcitonin levels. Among patients with SJS/TEN, disseminated intravascular coagulation was associated with a greater than 10-fold increase in mortality (78.1% vs 7%). LIMITATIONS: The study limitations include small sample size and a single hospital system. CONCLUSION: Disseminated intravascular coagulation is a potential complication of SJS/TEN and associated with higher mortality. Early recognition and appropriate management of this critical complication are important for patients with SJS/TEN.


Assuntos
Coagulação Intravascular Disseminada/etiologia , Coagulação Intravascular Disseminada/mortalidade , Hemorragia Gastrointestinal/complicações , Síndrome de Stevens-Johnson/complicações , Síndrome de Stevens-Johnson/mortalidade , Adulto , Idoso , Idoso de 80 Anos ou mais , Bacteriemia/complicações , Bacteriemia/microbiologia , Feminino , Humanos , Estimativa de Kaplan-Meier , Falência Hepática/complicações , Masculino , Pessoa de Meia-Idade , Insuficiência Renal/complicações , Insuficiência Respiratória/complicações , Taxa de Sobrevida
15.
Int J Dermatol ; 60(1): 53-59, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-33252832

RESUMO

BACKGROUND: To retrospectively review the outcomes of two rare cutaneous diseases, Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), and to question the practice of averaging the mortality rate on the assumption that they are one disease. METHODS: A retrospective chart review of all patients diagnosed with SJS and TEN by a dermatologist between January 1, 2000, and January 1, 2020, at our institution was performed. Seventy-one patients were identified (21 pediatric and 50 adults). Pathology slides from 32 adult patients (64%) were evaluated by a blinded board-certified dermatopathologist. RESULTS: Of the adult patients, 31 had SJS, two had SJS-TEN overlap, and 17 had TEN. All 21 patients in the pediatric group were diagnosed with SJS mainly caused by Mycoplasma. Mortality rates were 6.5% for SJS among adults and 35.3% for TEN. Chemotherapy-induced TEN is a trigger with 50% mortality. CONCLUSIONS: SJS was more common in adults and pediatric cases than TEN (3:1) and had a much better prognosis and outcome. Combining and averaging the mortality rates of TEN and SJS are not advised as SJS is mainly a mucocutaneous disorder with good prognosis versus TEN, a systemic toxicity of multiple organs with deep skin detachment.


Assuntos
Síndrome de Stevens-Johnson/etiologia , Síndrome de Stevens-Johnson/mortalidade , Adolescente , Adulto , Fatores Etários , Antibacterianos/efeitos adversos , Anti-Inflamatórios/uso terapêutico , Anticonvulsivantes/efeitos adversos , Criança , Pré-Escolar , Feminino , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Masculino , Pessoa de Meia-Idade , Infecções por Mycoplasma/complicações , Prognóstico , Estudos Retrospectivos , Índice de Gravidade de Doença , Esteroides/uso terapêutico , Síndrome de Stevens-Johnson/patologia , Síndrome de Stevens-Johnson/terapia , Adulto Jovem
17.
Allergol Int ; 70(2): 229-234, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33279401

RESUMO

BACKGROUND: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but life-threatening disorders characterized by widespread epidermal necrosis of the skin and mucosa. The severity-of-illness scoring system for TEN (SCORTEN) was widely used since 2000 as a standard prognostic tool consisting of seven clinical values. METHODS: To evaluate the prognosis using current treatments and risk factors for mortality, we retrospectively analyzed 59 cases of TEN, including SJS/TEN overlap treated in two university hospitals from January 2000 to March 2020. RESULTS: The mortality rate of TEN was 13.6% (8/59). All patients treated with high-dose steroid administration in combination with plasma exchange and/or immunoglobulin therapy recovered. Logistic regression analysis showed nine clinical composite scores, namely: heart rate (≧120 bpm), malignancy present, percentage of body surface area with epidermal detachment (>10%), blood urea nitrogen (>28 mg/dL), serum bicarbonate level (<20 mEq/L), serum glucose level (>252 mg/dL), age (≧71 years), the interval between disease onset and treatment initiation at the specialty hospital (≧8 days), and respiratory disorder within 48 h after admission. The receiver operating characteristic curves confirmed a high potential for predicting the prognosis of TEN. CONCLUSIONS: Recent developments in treatment strategies have contributed to the improved prognosis of TEN patients. A modified severity scoring model composed of nine scores may be helpful in the prediction of TEN prognosis in recent patients. Further large-scale studies are needed to confirm mortality findings to improve prognostication in patients with TEN.


Assuntos
Síndrome de Stevens-Johnson/mortalidade , Adolescente , Corticosteroides/uso terapêutico , Adulto , Idoso , Idoso de 80 Anos ou mais , Antibacterianos/efeitos adversos , Anti-Inflamatórios não Esteroides/efeitos adversos , Anticonvulsivantes/efeitos adversos , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Japão/epidemiologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Fatores de Risco , Índice de Gravidade de Doença , Síndrome de Stevens-Johnson/tratamento farmacológico , Adulto Jovem
18.
Rev. cuba. med ; 59(4): e53, oct.-dic. 2020. graf
Artigo em Espanhol | LILACS, CUMED | ID: biblio-1144510

RESUMO

Se estudiaron dos pacientes ingresados en sala de cuidados intensivos polivalente, los cuales presentaron necrolisis tóxica epidémica (NTE), complicación poco frecuente que tiene una incidencia de 1 a 4 casos por millón de habitantes. La causa de la enfermedad en estos pacientes fue medicamentosa y los principales medicamentos implicados fueron anfotericin B y ciprofloxacino. Una de las cuestiones que hace interesante el reporte de estas reacciones adversas es que se trata del primer caso de NTE relacionada con el uso de Anfotericin B Liposomal que se reporta en Cuba y en el resto de la comunidad científica. El diagnóstico se confirmó por biopsia de piel, ambos pacientes presentaron un riesgo de muerte, según índice de Scorten, mayor al 50 por ciento. Por lo infrecuente que es el tratamiento de esta afección en una sala de cuidados intensivos polivalentes, por lo raro que es ver esta enfermedad en relación a estos antibióticos antes mencionados, por lo dramático del cuadro clínico y por la evolución tórpida de los pacientes, se realizó esta investigación con el objetivo de transmitir la experiencia y el conocimiento a la comunidad médica y científica en general y a los profesionales que laboran en las salas de terapia intensiva polivalente en particular(AU)


Two patients admitted to a multipurpose intensive care ward were studied. They showed epidemic toxic necrolysis (NTE), a rare complication that has an incidence of 1 to 4 cases per million inhabitants. Drugs were the cause of the disease in these patients and the main drugs involved were amphotericin B and ciprofloxacin. This is the first case of NTE related to the use of Liposomal Amphotericin B reported in Cuba and in the rest of the scientific community, which makes it interesting. The diagnosis was confirmed by skin biopsy, both patients had risk of death, according to the Scorten index, higher than 50 percent. This research was carried out with the aim of communicating the experience and knowledge to the medical and scientific community in general and to the professionals who work in multipurpose intensive care rooms in particular, since this condition is rare in a multipurpose intensive care room, it is rare in relation to the aforementioned antibiotics, its dramatic clinical status and the torpid evolution of patients(AU)


Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Síndrome de Stevens-Johnson/mortalidade , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/mortalidade , Unidades de Terapia Intensiva , Cuba
19.
JAMA Dermatol ; 156(12): 1294-1299, 2020 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-33084873

RESUMO

Importance: Epidermal necrolysis is a rare severe cutaneous drug reaction associated with high mortality. The ABCD-10 score (age, bicarbonate, cancer, dialysis, 10% body surface area), a new prognostic score for mortality in epidermal necrolysis, was recently developed and validated in the US. However, to our knowledge, it remains to be externally validated in other cohorts. Objective: To assess ABCD-10 among patients in a contemporary Asian cohort and compare its performance with the Score of Toxic Epidermal Necrosis (SCORTEN) and study the associations of time and immunomodulatory therapy with the performance of ABCD-10 and SCORTEN. Design, Setting, and Participants: This retrospective cohort study was conducted over a 17-year period from January 2003 to March 2019 and included 196 patients with epidermal necrolysis who were recruited from Singapore General Hospital, the national referral center for epidermal necrolysis. Main Outcomes and Measures: In-hospital mortality. Discrimination and calibration of each risk score were assessed and compared using the area under the receiver operating characteristic curve and calibration plot, respectively. Results: Among 196 patients (median [interquartile range] age, 56 [42-70] years; 116 women [59.2%]), 45 (23.0%) did not survive to discharge. All risk factors in ABCD-10 were significantly associated with in-hospital mortality. However, dialysis before admission, the most heavily weighted factor in ABCD-10, performed weaker in this cohort (odds ratio, 3.7; 95% CI, 1.0-13.2, P = .04). Although the discrimination of ABCD-10 and SCORTEN did not differ (area under the curve: ABCD-10, 0.78; 95% CI, 0.72-0.85; vs SCORTEN, 0.77; 95% CI, 0.69-0.84; P = .53), the calibration of ABCD-10 was poorer compared with SCORTEN. From graphical analysis of the calibration plots, ABCD-10 showed mortality underestimation at lower score ranges and overestimation at higher score ranges. By contrast, SCORTEN was generally well calibrated, although at higher score ranges mortality may be overestimated. Assessment of calibration plots showed that there was increasing overestimation of mortality by SCORTEN during the later period or when immunomodulatory therapy was used compared with patients treated with supportive care alone. Calibration of ABCD-10 remained poor even during the later period or among patients treated with immunomodulatory therapy. Conclusions and Relevance: In this cohort of patients, the performance of SCORTEN was superior to ABCD-10 in mortality prognostication in epidermal necrolysis. However, it did display time-associated deterioration in calibration leading to overestimation of mortality risk. Future studies may consider revising the existing SCORTEN given its current good discrimination.


Assuntos
Índice de Gravidade de Doença , Síndrome de Stevens-Johnson/diagnóstico , Adulto , Idoso , Feminino , Mortalidade Hospitalar , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Curva ROC , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Síndrome de Stevens-Johnson/mortalidade , Síndrome de Stevens-Johnson/terapia
20.
J Burn Care Res ; 41(5): 945-950, 2020 09 23.
Artigo em Inglês | MEDLINE | ID: mdl-32498082

RESUMO

Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and Stevens-Johnson/TEN overlap syndrome (SJS/TEN) are severe exfoliative skin disorders resulting primarily from allergic drug reactions and sometimes from viral causes. Because of the significant epidermal loss in many of these patients, many of them end up receiving treatment at a burn center for expertise in the care of large wounds. Previous work on the treatment of this disease focused only on the differences in care of the same patients treated at nonburn centers and then transferred to burn centers. We wanted to explore whether patients had any differences in care or outcomes when they received definitive treatment at burn centers and nonburn centers. We queried the National Inpatient Sample database from 2016 for patients with SJS, SJS/TEN, and TEN diagnoses. We considered burn centers as those with greater than 10 burn transfers to their center and fewer than 5 burn transfers out of their center in a year. Multivariable logistic regression assessed factors associated with treatment at a burn center and mortality. Using the National Inpatient Sample, a total of 1164 patients were identified. These were divided into two groups, nonburn centers vs burn centers, and those groups were compared for demographic characteristics as well as variables in their hospital course and outcome. Patients treated at nonburn centers were more likely to have SJS and patients treated at burn centers were more likely to have both SJS/TEN and TEN. Demographics were similar between treatment locations, though African-Americans were more likely to be treated at a burn center. Burn centers had higher rates of patients with extreme severity and mortality risks and a longer length of stay. However, burn centers had similar actual mortality compared to nonburn centers. Patients treated at burn centers had higher charges and were more likely to be transferred to long-term care after their hospital stay. The majority of patients with exfoliative skin disorders are still treated at nonburn centers. Patients with SJS/TEN and TEN were more likely to be treated at a burn center. Patients treated at burn centers appear to have more severe disease but similar mortality to those treated at nonburn centers. Further study is needed to determine whether patients with these disorders do indeed benefit from transfer to a burn center.


Assuntos
Unidades de Queimados , Síndrome de Stevens-Johnson/terapia , Adulto , Idoso , Estudos de Coortes , Feminino , Hospitalização , Humanos , Masculino , Pessoa de Meia-Idade , Transferência de Pacientes , Padrões de Prática Médica , Estudos Retrospectivos , Fatores Socioeconômicos , Síndrome de Stevens-Johnson/diagnóstico , Síndrome de Stevens-Johnson/mortalidade , Taxa de Sobrevida
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