Epidermal necrolysis French national diagnosis and care protocol (PNDS; protocole national de diagnostic et de soins)

Ingen‐Housz‐Oro, Saskia; Duong, Tu-Anh; Bensaïd, Benoît; Bellon, N.; Prost, Nicolas de; Lu, Dévy; Lebrun‐Vignes, Bénédicte; Gueudry, J.; Béquignon, E.; Zaghbib, K.; Royer, G; Colin, A.; Do‐Pham, G.; Bodemer, Christine; Ortonne, Nicolas; Barbaud, A.; Fardet, L.; Chosidow, Olivier; Wolkenstein, Pierre

doi:10.1186/s13023-018-0793-7

Cited by 58 publications

(90 citation statements)

References 69 publications

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“…This decreased probability was also confirmed for patients with TEN ( P = 0·01). In the absence of proven effective specific treatment of EN, multidisciplinary supportive care, supposedly optimized in centres with EN experience and a specific interest in the disease, is the cornerstone of treatment . Considering the rarity of EN, a reference centre was created for this disease in 2005 in France, with several competence sites all over the French territory, to improve and standardize supportive care, teaching and research on the topic.…”

Section: Discussionmentioning

confidence: 99%

“…We cannot exclude that the lack of permanent dermatologists in the hospitals could cause a diagnostic delay. However, in France, thanks to the national reference centre for toxic bullous diseases implemented in 2005, actions were conducted to improve knowledge of the disease and diagnostic delay, such as dedicated national guidelines including an algorithm of transfer and facilities to ask for urgent advice by phone or e‐mail …”

Section: Discussionmentioning

confidence: 99%

“…Early recognition and diagnosis of EN as a severe cutaneous adverse reaction are key to the identification of culprit drugs, leading to better prognosis because early discontinuation of the culprit drug may decrease mortality . Without proven effective specific treatment, supportive care performed in dermatology departments, intensive care or burn units (according to the severity of the disease) remains the cornerstone of management.…”

mentioning

confidence: 99%

“…Without proven effective specific treatment, supportive care performed in dermatology departments, intensive care or burn units (according to the severity of the disease) remains the cornerstone of management. It includes skin antisepsis, local care of erosions with nonsticky dressings or white petroleum without removal of the detached epidermis, prevention of mucous membrane (especially ocular) fibrosis and symblepharons, prevention and treatment of infections, fluid resuscitation, prevention of hypothermia, nutritional support, management of acute respiratory failure and pain and psychological distress management . We hypothesized that, as in numerous domains of healthcare, physicians’ expertise of this rare condition is important, and consequently centres with experience of multidisciplinary management of EN can achieve these goals and thus reduce the risk of mortality.…”

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confidence: 99%

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Individual‐ and hospital‐level factors associated with epidermal necrolysis mortality: a nationwide multilevel study, France, 2012–2016

et al. 2019

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Summary Background Epidermal necrolysis (EN) is a rare and life‐threatening condition. Objectives To assess whether admitting hospital characteristics and interhospital transfer are associated with mortality due to EN. Methods We studied the French nationwide hospital discharge database (retrospective national cohort). All patients admitted during 2012–2016 with a code for EN in the International Classification of Diseases, 10th Revision, were eligible. We extracted data on the patients (age, sex, intensive care unit admission, comorbidities) and hospitals (private proprietary vs. public, nonteaching or teaching; and number of admissions for EN as a proxy for experience). Multivariable analysis was used to identify independent predictors of in‐hospital mortality with mixed logistic regression. Results We identified 991 patients (467 male; mean age 52·7 ± 23 years). They were admitted to 300 different hospitals, including teaching hospitals (25% of hospitals) for around half of the patients. Overall, 597 patients (60%) had a diagnosis of Stevens–Johnson syndrome (SJS), 171 (17%) had SJS/toxic epidermal necrolysis (TEN) overlap and 223 (23%) had TEN. In total, 109 (11%) patients died: nine (2%) with SJS, 26 (15%) with SJS/TEN overlap and 74 (33%) with TEN. The in‐hospital mortality rate was lower in centres with vs. without substantial EN experience – odds ratio for one supplemental EN admission in a department 0·5 (95% confidence interval 0·3–1·0); P = 0·05 – even after adjusting for potentially relevant individual risk factors. We found no significant association between mortality and interhospital transfer. Conclusions Our findings highlight increased survival of patients with EN in centres with a high volume of EN procedures. If confirmed in other settings, these findings reinforce the importance of expertise in early diagnosis and management of this condition. What's already known about this topic? Epidermal necrolysis (EN) is a rare and life‐threatening condition. At the individual level, risk factors for in‐hospital mortality have been identified. Few studies have examined the association between hospital characteristics and EN mortality, with special attention to referral hospitals. What does this study add? Short‐term mortality rates were lower for patients in centres with EN experience than in centres without EN experience, after adjusting for known risk factors. We found no association between interhospital transfer and survival. If confirmed in other settings, these findings support the early transfer of patients with suspected or diagnosed EN to centres with experience, where a multidisciplinary approach can be implemented by experienced healthcare professionals, to maximize short‐term survival.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

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confidence: 99%

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Individual‐ and hospital‐level factors associated with epidermal necrolysis mortality: a nationwide multilevel study, France, 2012–2016

et al. 2019

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“…Supportive care is the cornerstone of treatment. Patients with the most severe disease, requiring mechanical ventilation support, fluid resuscitation, and treatment of infectious complications, need care in an intensive care unit (ICU) or burn unit …”

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confidence: 99%

Health‐related quality of life and long‐term sequelae in survivors of epidermal necrolysis: an observational study of 57 patients

et al. 2019

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Summary Background Few studies have investigated the global burden of sequelae and health‐related quality of life (HRQoL) for survivors of epidermal necrolysis (EN). Objectives To investigate the long‐term HRQoL for survivors of EN using validated instruments. Methods We conducted a single‐centre study that enrolled patients who were admitted for EN between 2010 and 2017. HRQoL was assessed via phone interview using the Short Form (SF)‐36 questionnaire, Hospital Anxiety and Depression (HAD) scale, Impact of Event Scale‐Revised, and general quality‐of‐life outcomes, including EN‐specific sequelae. The primary outcome measure was the physical component summary (PCS) score of the SF‐36. Results In total, 57 survivors of EN [19 (33%) with intensive care unit (ICU) admission] were interviewed via telephone at a median of 3·6 years (1·9–6·1) after hospital discharge. The median PCS score was 0·44 SDs below that of the age‐ and sex‐matched reference population and was significantly lower for survivors of EN who were admitted to the ICU vs. those who were not [43·7 (28·7–49·3) vs. 51·2 (39·4–56·5), P = 0·042]. The proportion of patients with EN who had HAD‐anxiety score ≥ 8 or HAD‐depression score ≥ 5 was 54% and 21%, respectively. Physical and mental outcomes did not differ between patients with EN who were admitted to the ICU and survivors of septic shock. Reported EN‐specific sequelae were cutaneous (77%), ocular (70%), psychological (60%), dental/oral (49%), genital (30%) and respiratory (18%), with median intensity on a visual analogue scale. Conclusions Our study confirms the major burden and long‐term impact of EN on quality of life for survivors and emphasizes the need for prolonged close follow‐up after the acute phase. What's already known about this topic? Long‐term sequelae have been reported in 90% of survivors of epidermal necrolysis (EN). Few studies have investigated the global burden of sequelae and health‐related quality of life (HRQoL) in survivors of EN. What does this study add? Survivors of EN, particularly those admitted to the intensive care unit, had poorer physical HRQoL than the French reference population but had comparable HRQoL to survivors of septic shock. Survivors of EN exhibited symptoms of anxiety, depression and post‐traumatic stress syndrome. The most frequent sequelae were cutaneous, ocular and psychological, with visual analogue scale scores of 5/10 and 6/10. These results confirm the burden of EN on quality of life.

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Assessment of Treatment Approaches and Outcomes in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

et al. 2021

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syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe drug reactions associated with a high rate of mortality and morbidity. There is no consensus on the treatment strategy.OBJECTIVE To explore treatment approaches across Europe and outcomes associated with the SJS/TEN disease course, as well as risk factors and culprit drugs. DESIGN, SETTING, AND PARTICIPANTSA retrospective pan-European multicenter cohort study including 13 referral centers belonging to the ToxiTEN ERN-skin subgroup was conducted. A total of 212 adults with SJS/TEN were included between January 1, 2015, and December 31, 2019, and data were collected from a follow-up period of 6 weeks. MAIN OUTCOMES AND MEASURESRisk factors for severe acute-phase complications (acute kidney failure, septicemia, and need for mechanical ventilation) and mortality 6 weeks following admission were evaluated using a multivariable-adjusted logistic regression model. One tool used in evaluation of severity was the Score of Toxic Epidermal Necrolysis (SCORTEN), which ranges from 0 to 7, with 7 the highest level of severity. RESULTSOf 212 patients (134 of 211 [63.7%] women; mean [SD] age, 51.0 [19.3] years), the mean (SD) body surface area detachment was 27% (32.8%). In 176 (83.0%) patients, a culprit drug was identified. Antibiotics (21.2%), followed by anticonvulsants (18.9%), nonsteroidal anti-inflammatory drugs (11.8%), allopurinol (11.3%), and sulfonamides (10.4%), were the most common suspected agents. Treatment approaches ranged from best supportive care only (38.2%) to systemic glucocorticoids (35.4%), intravenous immunoglobulins (23.6%), cyclosporine (10.4%), and antitumor necrosis factor agents (3.3%). Most patients (63.7%) developed severe acute-phase complications. The 6-week mortality rate was 20.8%. Maximal body surface area detachment (Ն30%) was found to be independently associated with severe acute-phase complications (fully adjusted odds ratio [OR], 2.49; 95% CI, 1.21-5.12; P = .01) and SCORTEN greater than or equal to 2 was significantly associated with mortality (fully adjusted OR, 10.30; 95% CI, 3.82-27.78; P < .001). Cyclosporine was associated with a higher frequency of greater than or equal to 20% increase in body surface area detachment in the acute phase (adjusted OR, 3.44; 95% CI, 1.12-10.52; P = .03) and an increased risk of infections (adjusted OR, 7.16; 95% CI, 1.52-33.74; P = .01). Systemic glucocorticoids and intravenous immunoglobulins were associated with a decreased risk of infections (adjusted OR, 0.40; 95% CI, 0.18-0.88; P = .02). No significant difference in 6-week mortality was found between treatment groups.CONCLUSIONS AND RELEVANCE This cohort study noted differences in treatment strategies for SJS/TEN in Europe; the findings suggest the need for prospective therapeutic studies to be conducted and registries to be developed.

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Epidermal necrolysis French national diagnosis and care protocol (PNDS; protocole national de diagnostic et de soins)

Cited by 58 publications

References 69 publications

Individual‐ and hospital‐level factors associated with epidermal necrolysis mortality: a nationwide multilevel study, France, 2012–2016

Individual‐ and hospital‐level factors associated with epidermal necrolysis mortality: a nationwide multilevel study, France, 2012–2016

Health‐related quality of life and long‐term sequelae in survivors of epidermal necrolysis: an observational study of 57 patients

Assessment of Treatment Approaches and Outcomes in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

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