Physical and Psychological Recovery Following Toxic Epidermal Necrolysis: A Patient Survey.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are acute, life-threatening diseases that cause sloughing of the skin and mucous membranes. Despite improved survival rates, few studies focus on long-term outcomes. We conducted a single-center review of all patients with SJS/TEN admitted from January 2008 to 2014. SJS/TEN survivors were invited to participate in the validated Veterans RAND 12-Item Health Survey (VR-12) to assess health-related quality of life using a mental health composite score and physical health component score (PCS). The sample was compared to U.S. norms using one-sample two-tailed t tests. A second questionnaire addressed potential long-term medical complications related to SJS/TEN. Of 81 treated subjects, 24 (30%) long-term survivors responded. Participants identified cutaneous sequelae most frequently (79%), followed by nail problems (70%), oral (62%), and ocular (58%) sequalae. Thirty-eight percent rated their quality of life to be "unchanged" to "much better" since their episode of SJS/TEN. The average PCS was lower than U.S. population norms (mean: 36 vs 50, P = .006), indicating persistent physical sequelae from SJS/TEN. These results suggest that SJS/TEN survivors continue to suffer from long-term complications that impair their quality of life and warrant ongoing follow-up by a multidisciplinary care team.

Understanding Epidermal Necrolysis after the Initial Hospitalization.

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.

Post-traumatic stress disorder in Stevens-Johnson syndrome and toxic epidermal necrolysis: prevalence and risk factors. A prospective study of 31 patients.

BACKGROUND: Epidermal necrolysis is a rare and severe cutaneous adverse reaction to drugs with long-term somatic consequences and potentially underrecognized psychological complications. OBJECTIVES: To assess the prevalence and risk factors of post-traumatic stress disorder (PTSD) in Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) in a population of adults undergoing psychiatric evaluation. METHODS: In this prospective study, we included adult patients admitted at the acute phase of SJS/TEN to our dermatology department from June 2009 to February 2013. The main objective was to assess the prevalence of PTSD at 6 months after the acute disease phase, defined by a PTSD Checklist score > 44. Secondary objectives were to investigate risk factors of PTSD in the medical history of patients and characteristics of the disease at the acute phase by the Peritraumatic Dissociative Experience Questionnaire (PDEQ) and Peritraumatic Distress Inventory (PDI) and the degree of impairment on the Sheehan Disability Scale. RESULTS: We initially included 32 of 80 patients admitted during the study period. At 6 months, seven of 30 still followed up had a PTSD Checklist score > 44, suggesting a PTSD prevalence of 23%; 23 (77%) patients had a hydroxyzine prescription at the acute phase. The main risk factors associated with PTSD at 6 months were psychological results at the acute phase. CONCLUSIONS: Despite frequent prescription of hydroxyzine at the acute phase, almost one-quarter of patients with SJS/TEN had PTSD at 6 months. A systematic psychiatric evaluation should be offered regularly for at least 1 year after the acute disease phase.

Cost-effectiveness of osteo-odonto keratoprosthesis in Singapore.

PURPOSE: To determine the long-term cost-effectiveness of osteo-odonto keratoprosthesis (OOKP) relative to no treatment among patients with end-stage corneal and ocular surface diseases in Singapore. DESIGN: Cost-effectiveness analysis based on data from a retrospective cohort study. METHODS: From a health system perspective, we calculated the incremental cost-effectiveness ratio of OOKP treatment relative to no treatment over a 30-year horizon, based on data from a cohort of 23 patients who underwent OOKP surgery between 2004 and 2009 at Singapore National Eye Centre. Preoperative and postoperative vision-related quality-of-life values were estimated from patients' visual outcomes and were used to calculate the gain in quality-adjusted life years (QALYs) resulting from OOKP treatment. Unsubsidized costs for surgery, consultations, examinations, medications, follow-up visits, and treatments for complications were retrieved from patients' bills to estimate the total costs associated with OOKP treatment. Sensitivity analyses were conducted to test the robustness of the model. RESULTS: Over a 30-year period, OOKP treatment, compared with no treatment, improved QALYs by 3.991 among patients with end-stage corneal and ocular surface diseases at an additional cost of S$67 840 (US$55 150), resulting in an incremental cost-effectiveness ratio of S$17 000/QALY (US$13 820/QALY). CONCLUSIONS: Based on commonly cited cost-effectiveness benchmarks, the OOKP is a cost-effective treatment for patients with end-stage corneal and ocular surface diseases.

Internet accounts of serious adverse drug reactions: a study of experiences of Stevens-Johnson syndrome and toxic epidermal necrolysis.

BACKGROUND: Life-threatening adverse drug reactions (ADRs) such as Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) continue to affect patients' lives long after the event. Survivors and their relatives rely heavily on Internet sources for support and advice, but narratives of their experiences posted on patient websites have not been explored previously. OBJECTIVES: The aim of the study was to illuminate patient experience by analysing Internet narratives of drug-induced SJS and TEN and considering the reasons for postings on patient websites, and the concerns they reflect. We also aimed to compare these experiences with a previous study of survivors of SJS and TEN conducted by face-to-face interview. METHODS: We searched for unsolicited personal narratives or descriptions of drug-induced SJS and TEN posted on the Internet between February 2009 and June 2010, and analysed them using a thematic qualitative approach. RESULTS: We analysed 208 Internet descriptions. Motivation for posting on the Internet included a desire to share experiences and to seek advice from others. Patients and their relatives expressed concern that the ADR may be hereditary, worries about effects on fertility and a fear of recurrence. They also wished to increase awareness of the potential harms from medicines and to inform others of the suspected cause of the ADR. CONCLUSION: Individuals experiencing SJS or TEN had many unanswered questions and concerns long after the event. Our findings could guide health professionals in the management of survivors of the ADR, and in communicating more effectively with patients and their relatives. Internet forum postings of patient experiences of ADRs provide insight into patient concerns and supplement findings from detailed face-to-face interviews.

Analysis of body constitution of fifty-two patients with Stevens-Johnson Syndrome (SJS) using Kampo Medical Questionnaires: prediction of SJS based on body constitution using decision tree.

UNLABELLED: Reports have indicated a relationship between adverse drug reaction (ADR) and Human Leukocyte Antigen (HLA) polymorphism and a relationship between Body Constitution (BC) and HLA polymorphism. Thus, a relationship between ADR and BC is suggested. We therefore created a questionnaire (hereinafter "Questionnaire") to survey the typical BC of Stevens-Johnson Syndrome (SJS) patients to determine how they differ from healthy persons, and studied the relationship between the development of SJS and BC. The Questionnaire had 30 typical items selected from those relevant to the BC necessary for the diagnosis and therapy of Sho-syndrome in Kampo Medicine. In the comparison of the prevalence of BCs between SJS patients and control persons, the prevalence of three BCs in the SJS group was significantly higher than that in the control group: 1) Does your throat ever feel closed up? ANSWER: Yes, 2) Do you easily feel hot flashes or burning cheeks even though your hands and feet feel cold? ANSWER: Yes, and 3) Do your lips or gums look dull red? ANSWER: Yes. In the analysis using the decision tree, the concentrated group of SJS patients (eighty-fold) was extracted using two decision trees consisting of 3 index variables. Persons with BCs from any of 1) to 3) are suggested to be at high risk of developing SJS.

Patient experiences of serious adverse drug reactions and their attitudes to medicines: a qualitative study of survivors of Stevens-Johnson syndrome and toxic epidermal necrolysis in the UK.

BACKGROUND: Adverse drug reactions (ADRs) cause significant morbidity and mortality and account for around 6.5% of hospital admissions. Patient experiences of serious ADRs and their long-term impact on patients' lives, including their influence on current attitudes towards medicines, have not been previously explored. OBJECTIVE: The aim of the study was to explore the experiences, beliefs, and attitudes of survivors of serious ADRs, using drug-induced Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) as a paradigm. METHODS: A retrospective, qualitative study was undertaken using detailed semi-structured interviews. Fourteen adult survivors of SJS and TEN, admitted to two teaching hospitals in the UK, one the location of a tertiary burns centre, were interviewed. Interview transcripts were independently analysed by three different researchers and themes emerging from the text identified. RESULTS: All 14 patients were aware that their condition was drug induced, and all but one knew the specific drug(s) implicated. Several expressed surprise at the perceived lack of awareness of the ADR amongst healthcare professionals, and described how the ADR was mistaken for another condition. Survivors believed that causes of the ADR included (i) being given too high a dose of the drug; (ii) medical staff ignoring existing allergies; and (iii) failure to monitor blood tests. Only two believed that the reaction was unavoidable. Those who believed that the condition could have been avoided had less trust in healthcare professionals. The ADR had a persisting impact on their current lives physically and psychologically. Many now avoided medicines altogether and were fearful of becoming ill enough to need them. CONCLUSIONS: Life-threatening ADRs continued to affect patients' lives long after the event. Patients' beliefs regarding the cause of the ADR differed, and may have influenced their trust in healthcare professionals and medicines. We propose that clear communication during the acute phase of a serious ADR may therefore be important.

Biological skin covers in treatment of two cases of the Lyell's syndrome.

The treatment of two cases of toxic epidermal necrolysis (Lyell's syndrome) is described. Although some features were common for both ones (young men practically of the same age, reaction after using the same drug) the clinical course of illness was very different. Spontaneous epithelisation of partial-thickness lesions and definitive healing under the xenografts in one patient and full-thickness skin-loss on 12% of body surface with severe septic complications requiring application of cultured keratinocytes and/or skin autografting in the other patient were the main differences. The interdisciplinary approach using a burns treatment protocol in non-burned patient including the close co-operation with the tissue bank in preparing different types of biological covers has been applied.

[Electroconvulsive therapy in treatment of acute life threatening catatonia in toxic epidermal necrolysis (Lyell syndrome)]. / Elektrokonvulsionstherapie zur Behandlung der akuten lebensbedrohlichen Katatonie bei toxischer epidermaler Nekrolyse (Lyell-Syndrom).

Toxic epidermal necrolysis (Lyell syndrome) developed in a 43-year woman treated with carbamazepine, perazine, and sulpiride for recurrent manic bipolar disorder. Five days after withdrawal of all psychotropic medication her psychiatric illness was exacerbated and a syndrome of lethal catatonia developed which was promptly treated with unilateral electroconvulsive therapy (ECT). Rapid remission of lethal catatonia after the first ECT treatment and of her affective disorder within days was achieved. According to clinical observations of the preneuroleptic era, early improvement of lethal catatonia following ECT may have been supported by concurrent high dose corticosteroid therapy for toxic epidermal necrolysis.

Toxic epidermal necrolysis: a medical student's perspective.

As a third year medical student, I was hospitalized for approximately one month with Toxic Epidermal Necrolysis (TEN). Consequently, I have developed an insight into the role of patient as well as that of medical care giver. My experience prompted an intense interest in this particular adverse drug reaction and research into treatment recommendations. Treatment has changed in recent years and this resulted in significantly improved survival. Steroids, once commonly used, are now considered contraindicated. Because of the wide variety of medications which may be associated with this adverse reaction, it is essential to be familiar with the clinical presentation of TEN, as well as the initial steps in treatment.