Treatment Of Stevens-Johnson Syndrome Research Articles

A bioinformatic approach to identify pathogenic variants for Stevens-Johnson syndrome.

Stevens-Johnson syndrome (SJS) produces a severe hypersensitivity reaction caused by Herpes simplex virus or mycoplasma infection, vaccination, systemic disease, or other agents. Several studies have investigated the genetic susceptibility involved in SJS. To provide further genetic insights into the pathogenesis of SJS, this study prioritized high-impact, SJS-associated pathogenic variants through integrating bioinformatic and population genetic data. First, we identified SJS-associated single nucleotide polymorphisms from the genome-wide association studies catalog, followed by genome annotation with HaploReg and variant validation with Ensembl. Subsequently, expression quantitative trait locus (eQTL) from GTEx identified human genetic variants with differential gene expression across human tissues. Our results indicate that two variants, namely rs2074494 and rs5010528, which are encoded by the HLA-C (human leukocyte antigen C) gene, were found to be differentially expressed in skin. The allele frequencies for rs2074494 and rs5010528 also appear to significantly differ across continents. We highlight the utility of these population-specific HLA-C genetic variants for genetic association studies, and aid in early prognosis and disease treatment of SJS.

Biologic TNF-α Inhibitors for Stevens-Johnson Syndrome, Toxic Epidermal Necrolysis, and TEN-SJS Overlap: A Study-Level and Patient-Level Meta-Analysis.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe cutaneous adverse reactions with high morbidity and mortality and not clearly established treatment protocol. This meta-analysis aimed to evaluate the efficacy and safety of three biologic TNF-α inhibitors (infliximab, etanercept, adalimumab) in the treatment of SJS, SJS-TEN overlap, and TEN. Electronic databases were searched for original studies containing human participants diagnosed with SJS/TEN and treated with biologic TNF-α inhibitors. Individual patient data were collected and summarized to provide a comprehensive overview on therapeutic efficacy of different biologic TNF-α inhibitors for SJS, SJS-TEN overlap, and TEN, respectively. Meta-analyses on aggregated study data were conducted using random-effects model. Overall, 55 studies with 125 sets of individual patient data were included. Infliximab was used to treat 3 patients with SJS-TEN overlap and 28 patients with TEN, and the actual mortality rate was 33.3% and 17%, respectively. Etanercept was administered to 17 patients with SJS, 9 patients with SJS-TEN overlap, and 64 patients with TEN, and mortality rate was reported to be 0%, 0%, and 12.5%, respectively. For participants with TEN, no significant difference was found in time of reepithelialization, hospitalization time, and mortality rate comparing etanercept with infliximab. More sequelae were reported in patients receiving infliximab than in patients treated with etanercept (39.3% versus 6.4%). Adalimumab was administered to four patients with TEN, and mortality rate was 25%. Meta-analyses on aggregated study data revealed significantly shortened hospitalization time in etanercept compared with non-etanercept groups [weighted mean differences (WMD) -5.30; 95% confidence interval (CI) -8.65 to -1.96]. Etanercept was associated with a survival benefit for patients when compared with non-etanercept treatment, however, the analysis was not statistically significant (odds ratio 0.55; 95% CI 0.23-1.33). On the basis of the current findings, etanercept is currently the most promising biologic therapy for SJS/TEN. Further evaluation in prospective studies is required to confirm its efficacy and safety.

A comparison of international treatment guidelines for Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis.

Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are rare and life-threatening mucocutaneous diseases that occur almost exclusively as a result of adverse drug reactions, although there are rare cases attributed to infection, immunization, or malignancy.1,2 Given the low incidence of these diseases as well as the high level of morbidity and mortality, randomized controlled clinical trials are difficult to perform, making it difficult to establish a "gold-standard" treatment. To date, there are only five published articles in the literature detailing evidence-based guidelines for the treatment of SJS and TEN, one of which is specifically tailored to pediatric and young adult patients.3-7 These guidelines have significant overlap in regards to the importance of prompt discontinuation of the offending drug and the need for supportive care, but there are differences in regards to the ideal supportive care measures. Additionally, there is still no clear consensus agreement on the pharmacological treatment of SJS and TEN.4-7 Herein, we aim to compare the international treatment guidelines for management of SJS and TEN as well as promote continued discussion and a multidisciplinary approach to establish consensus recommendations for these mucocutaneous emergencies.

Pembrolizumab-induced Stevens-Johnson syndrome in advanced squamous cell carcinoma of the lung: A case report and review of literature

BACKGROUNDFor advanced lung squamous cell carcinoma, immune checkpoint inhibitors (ICIs) have been regarded as one of the optimal therapies. While immune-related adverse events (irAEs) are common in ICI treatment, cutaneous toxicities are among the most common irAEs. Most immune-related skin toxicity grades are low, and the prognosis is good. However, Stevens-Johnson syndrome (SJS) is a rare but extremely severe cutaneous adverse drug reaction with high mortality.CASE SUMMARYWe report a rare case of SJS induced by pembrolizumab. The case involved a 68-year-old female who was diagnosed with advanced squamous cell carcinoma of the lung. SJS appeared after one cycle of immunotherapy combined with chemotherapy. After treatment with prednisone hormone symptoms, anti-infection, gamma globulin, and antipruritic agents, the skin toxicity of the patients gradually decreased and eventually disappeared. Although the antitumor treatment was stopped due to serious adverse reactions, the tumor of the patient remained stable for nearly half a year after one cycle of immune therapy combined with chemotherapy, which also corroborates the delayed effect of immunotherapy.CONCLUSIONWe believe our report can provide some references for the treatment of SJS and the treatment of immune-related adverse reactions.

Combined use of cyclosporine in the treatment of Stevens-Johnson syndrome/toxic epidermal necrolysis.

The exact efficacy of cyclosporine in the treatment of Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) still needs evidence from more clinical data. This study was designed to compare the effectiveness and side-effects of combined use of cyclosporine in the treatment TEN with glucocorticoids (GC)/i.v. immunoglobulin G (IVIG). A total of 46 patients with SJS/TEN were enrolled and classified into two groups based on the therapeutic drugs used. Clinical characteristics, interventions, outcomes, and disease progressions were collected and compared between the two groups. In our cohort, seven patients eventually died and the overall fatality rate was 15.2%, but there was no difference between the two groups (p=0.557). On discharge, the median SCORe of Toxic Epidermal Necrosis (SCORTEN) fell from 2.0 at admission to 1.0 and the median body surface area detached fell from 32.0% at admission to 9.5%. Patients in the cyclosporine group had a higher rate of re-epithelialized area than patients in the non-cyclosporine group (p < 0.05). Cyclosporine significantly reduced the length of stay (19.0 vs. 13.0 days, p=0.019) and the rate of systemic infection (71.4% vs. 36.0%, p=0.017) compared with the non-cyclosporine group. SCORTEN was the only significant risk factor for death and the risk ratio was 1.96 (1.17-3.31, p=0.011). Conclusively, the combined use of cyclosporine could reduce the occurrence of systemic infection and accelerate the re-epithelialization.

Systemic interventions for treatment of Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and SJS/TEN overlap syndrome.

Systemic interventions for treatment of Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and SJS/TEN overlap syndrome.

Механізми етіопатогенезу, клініки та сучасні підходи до лікування синдрому стівенса-джонсона та токсичного епідермального некролізу

Abstract. This article contains descriptive data on the etiology, pathogenesis, clinical presentation, diagnostics and treatment of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). SJS and TEN are relatively rare conditions, however, due to their severity, lethality and long-term negative consequences it is not only necessary to understand their etiopathogenesis clearly, but it’s also crucial to be alert to the patient’s allergic anamnesis for timely assessment of their condition and diagnosis with following adequate therapy. Prevalence of these diseases is from 0.4 to 1.4 cases per million persons per year, total lethality is approximately — 20 %. Development of these diseases is related to genetic predisposition of the human leukocyte antigen system (HLA). Medications are the most common triggers and play an important role in their onset, other factors, including infections, can also significantly affect the risk of their inception. In the present day, the SCORTEN scale is used to assess the severity of SJS and TEN. General principles of patients’ curation are based on the multidisciplinary approach, early diagnosis, discontinuation of trigger medications that could possibly cause SJS and TEN. In case when SJS/TEN were caused by infections, patient receives appropriate etiotropic therapy to eliminate the infection. The complex treatment algorithm must ensure hemodynamic stability, pain reduction, detoxification, local treatment, elimination of concomitant infections, use of anti-inflammatory and various immunotropic agents.

Blepharoconjunctivitis as Manifestation of Stevens-Johnson syndrome

Background: Stevens Johnson syndrome (SJS) is a collection of acute and life threatening skin symptoms caused by an allergic drug reaction. SJS also attacks the other mucous membranes, one of which is the eye. SJS is a rare case. The initial complaint of SJS is the eruption on the skin in the form of redness followed by blisters and attacking other mucous membranes. The ocular manifestations of SJS are conjunctivitis, corneal erosion, corneal ulcers, and blepharitis. Methods: Treatment of SJS’s ocular manifestation is administration of drugs and surgical intervention. Administration of drugs that often done in the acute phase is antibiotic eye drops with steroids to prevent complications. Complications that can occur include symblepharon, districhiasis, and keratitis. This complication can cause a sharp decrease in vision. Results: This case report discusses a 68 year old male patient with blisters all over his body accompanied by swelling in both eyes, redness in both eyes, and difficult to open eyes. Patients with history of taking the new drug a month ago. No history of drug allergy. The patient suffered from hypertension, non-hemorrhagic stroke, and epilepsy with irregular treatment. Patient was performed a fluorescein test. In this case, the patient is given antibiotic ointment and artificial tears with steroids. In this case there were no complications. Conclusion: The ocular manifestations of SJS if handled quickly and properly can prevent long-term complications.

Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Review of Diagnosis and Management.

Stevens–Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are rare diseases that are characterized by widespread epidermal necrosis and sloughing of skin. They are associated with significant morbidity and mortality, and early diagnosis and treatment is critical in achieving favorable outcomes for patients. In this scoping review, Excerpta Medica dataBASE and PubMed were searched for publications that addressed recent advances in the diagnosis and management of the disease. Multiple proteins (galectin 7 and RIP3) were identified that are promising potential biomarkers for SJS/TEN, although both are still in early phases of research. Regarding treatment, cyclosporine is the most effective therapy for the treatment of SJS, and a combination of intravenous immunoglobulin (IVIg) and corticosteroids is most effective for SJS/TEN overlap and TEN. Due to the rare nature of the disease, there is a lack of prospective, randomized controlled trials and conducting these in the future would provide valuable insights into the management of this disease.

Clinical Aspects of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis With Severe Ocular Complications in Taiwan.

Purpose: Over the last decade, there has been tremendous progress in the treatment of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). To understand whether this has resulted in better ophthalmic outcomes, we aimed to study the incidence of severe ocular complications (SOCs) in the acute and chronic stage among SJS/TEN patients, major causative medications, and therapeutic effect of medical and surgical treatment.Methods: Using electronic medical records review of patients of Chang Gung Memorial Hospital Linkou Branch from 2010 to 2020, 119 patients (236 eyes) received ophthalmic consultation during the acute stage and were retrospectively studied. Sotozono's grading score systems for acute and chronic SJS/TEN were employed for accessing correlation between acute and chronic presentations, the therapeutic effect of systemic etanercept treatment, and outcome of early amniotic membrane transplantation (AMT) performed in patients with severe acute SOCs.Results: There were 46 male and 73 female patients with a mean age of 45.6 ± 22.7 years old (2–90 years), and follow-up time of 408.3 ± 351.0 (116–1,336) days. The numbers of patients with SJS, overlap syndrome, and TEN were 87, 9, and 23, respectively. In total, 109 eyes (55 patients) had acute SOCs, which comprised 46.2% of patients who underwent ophthalmic examination. Antiepileptics were the most common category of culprit drugs causing SOCs in the acute stage. At the end of follow-up, there were 14 eyes (9 patients) with chronic SOCs (5.9%), and non-steroidal anti-inflammatory drugs and cold medicine were the most common causative medications that were associated with severe chronic sequela. The correlation between Sotozono's acute and chronic grading score showed a positive relationship [Spearman's rank correlation coefficient (r) = 0.52, p < 0.001]. The average chronic grading scores in patients receiving systemic corticosteroid combined with etanercept treatment were significantly lower than those receiving corticosteroid only, Finally, the average chronic grading scores in patients receiving AMT <7 days after onset were significantly lower than those performed beyond 7 days.Conclusion: Our study implies that acute manifestation can be an indicator for chronic sequelae. Additional early etanercept treatment and early AMT showed beneficial effect in reducing chronic ocular sequela.

Complete Fusion of Both Eyelids in Stevens-Johnson Syndrome: Case Report

Abstract An 18-year-old woman developed Stevens-Johnson syndrome (SJS) with ocular involvement after taking ibuprofen. She was admitted to another hospital, received saline flushes and bacitracin ophthalmic ointment to the eyes, and became unable to open them. Upon transfer to this burn center 3 weeks after symptom onset, there was complete fusion of both eyelids with no visible cornea or sclera. She underwent bilateral operative scar release. After opening the lids, meticulous debridement of cicatricial membranes and release of symblephara were performed with subsequent placement of amniotic membrane grafts. Her vision slowly improved, though her long-term visual prognosis remains guarded. Early recognition and treatment of SJS or toxic epidermal necrolysis (TEN) with ocular involvement is imperative. Even mild cases may require intensive topical lubrication, steroids, and antibiotics, with early placement of amniotic membrane grafts in severe cases. Prompt intervention and daily evaluation are paramount in preventing lifelong visual disability.

Retrospective Study of Patients With SJS/TEN Treated at a Tertiary Burn Unit in Canada: Overview of 17 Years of Treatment

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are adverse drug reactions. To learn about the clinical characteristics of patients with SJS/TEN including treatments provided, outcomes, and mortality. We conducted a retrospective chart review of patients who were hospitalized with the diagnosis of SJS/TEN at the Ross Tilley Burn Center between the years 1999 and 2015. A total of 43 patients were identified with a mean age of 54 ± 19 (58, 18-85). The most common offending medications were allopurinol and carbamazepine. The overall mortality rate in our study is 21% with the most common causes of death being multiorgan failure and sepsis. The majority of our patients had oral (84%), ocular (79%), and genital (60%) involvement during hospitalization. Our data revealed that combination treatment involving oral corticosteroids with intravenous immunoglobulin (IVIG) had the highest mortality rate in our study since 55% (6/11) of patients who were treated in this manner passed away compared to 11% (2/18) of patients passing away who were treated with solely IVIG and 33% (1/3) who were treated with only supportive care. Our study also demonstrates the addition of etanercept and cyclosporine treatment in the second time period we studied: 2008-2015 versus the earlier time period of 1999-2007. None of the patients in our study who were treated with therapies including cyclosporine and/or etanercept passed away. Our study sheds light on a possible beneficial role of cyclosporine and etanercept for the treatment of SJS and TEN and reinforces the necessity of a multidisciplinary care team for patients.

Differences in Treatment of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis at Burn Centers and Nonburn Centers.

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.

Stevens-Johnson syndrome and toxic epidermal necrolysis in pregnant patients: A systematic review.

BackgroundStevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are two of the most severe dermatologic emergencies. Although pregnant women comprise a subset of individuals at risk for SJS and TEN development, little is known with regard to outcomes and treatment.ObjectiveThis study aimed to conduct a systematic review to characterize the risk factors, outcomes, and treatment of SJS and TEN in pregnant patients and newborns.MethodsA primary literature search was conducted using PubMed in September 2019, using the following search terms entered in separate pairs: pregnant or pregnancy and stevens-johnson or SJS or toxic epidermal necrolysis. Reviews, studies in a language other than English, and articles not including pregnant patients were excluded.ResultsTwenty-six articles were included for review, including a total of 177 patients. The average maternal age for a reaction was 29.9 years, gestational age was 24.9 weeks, and time to reaction after drug initiation was 27.5 days. Approximately 85% of pregnant women in this review were infected with HIV. The most common causative medications were antiretroviral therapy (90% of all cases), antibiotics (3%), and gestational drugs (2%). Of the 94 cases in which outcome data were available, the survival rates of pregnant women and newborns after delivery were 98% and 96%, respectively. Withdrawal of the offending agent and supportive care was often sufficient for treatment, but antibiotics, steroids, and intravenous immunoglobulin were implemented in some cases. Complications included preterm labor, vaginal stenosis, and vaginal adhesions.ConclusionGiven the predominance of studies focusing on the subset of pregnant patients who are infected with HIV, SJS and TEN is most commonly reported in young patients after antiretroviral therapy, primarily nevirapine. Overall mortality is lower than that of the general population, but similar to the expected mortality rates of younger adults. Early recognition and withdrawal of the offending agent is essential to mitigate the distinct consequences of these conditions in the pregnant population.

549 Differences in Treatment of Stevens-johnson Syndrome and Toxic Epidermal Necrolysis at Burn Centers and Non-burn Centers

Abstract Introduction Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and SJS/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 non-burn 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 non-burn centers. Methods We queried the National Inpatient Sample (NIS) database from 2016 for patients with SJS, SJS/TEN, and TEN diagnosis. 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. Results Using the NIS, a total of 1164 patients were identified. These were divided into two groups, non-burn 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 non-burn 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 non-burn centers. Patients treated at burn centers had higher charges and were more likely to be transferred to long-term care after their hospital stay. Conclusions The majority of patients with exfoliative skin disorders are still treated at non-burn 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 non-burn centers. Our data suggest that more patients with these disorders could benefit from transfer to a burn center. Applicability of Research to Practice These data may aid in outreach efforts to improve appropriate transfers of patients with severe exfoliative skin disorders to burn centers for definitive care.

760 Patients with Pre-existing Mental Illness Who Developed Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: Lessons Learned at a Large Tertiary Burn Center and the Importance of Accurately Diagnosing Psychiatric Disorders

Abstract Introduction In recent years, burn centers are managing more patients with exfoliative skin disorders including Stevens-Johnson Syndrome (SJS), Toxic Epidermal Necrolysis (TEN), and SJS/TEN overlap. While it is well known that burn patients have higher rates of co-morbid psychiatric disorders than the general population, the incidence of pre-existing psychiatric disorders among patients who develop SJS, SJS/TEN overlap, and TEN is unknown. This study aimed to characterize a cohort of patients with pre-existing psychiatric disorders admitted to a tertiary burn center for treatment of SJS, SJS/TEN overlap, and TEN with specific focus on those who received the offending agent for a psychiatric indication. Methods A retrospective descriptive case series using an institutional burn center registry was performed. All patients admitted to a single verified burn center between January 1, 2009 and December 31, 2018 with biopsy-proven SJS, SJS/TEN overlap, or TEN and the presence of a co-morbid psychiatric disorder were identified. Demographic, hospital, and clinical information were extracted from the burn registry and verified through review of the electronic medical record. Results Among 168 patients with biopsy-proven SJS, SJS/TEN overlap, or TEN, 18% (30/168) had a pre-existing psychiatric disorder, with the offending agent being prescribed for a psychiatric indication in 30% (10/30) of patients. Lamotrigine was the offending agent in 80% of cases and prescribed 100% of the time for a psychiatric indication. Of those who received lamotrigine, patients were 100% female, 63% black, and had an average age of 38 years. The mean length of stay was 24 days and 88% received a psychiatric consultation. While 75% of patients were started on lamotrigine for a diagnosis of bipolar disorder, none of these patients met criteria for bipolar disorder. Conclusions Pre-existing psychiatric comorbidity is less common among patients that develop SJS, SJS/TEN overlap, and TEN than in burn-injured patients. For patients with pre-existing psychiatric disorders who develop these potentially fatal skin diseases from an offending agent that was prescribed for a psychiatric indication, early involvement of psychiatry colleagues is recommended to ensure proper psychiatric diagnosis and management moving forward. Applicability of Research to Practice This study highlights the importance of accurate assessment for and diagnosis of bipolar disorder prior to determining treatment approach. For the burn surgeon treating these patients, early involvement of psychiatric consultants is recommended and extremely important.

Systemic interventions for treatment of Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and SJS/TEN overlap syndrome

Systemic interventions for treatment of Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and SJS/TEN overlap syndrome

The Role of Biological Skin Substitutes in Stevens-Johnson Syndrome: Systematic Review.

Stevens-Johnson syndrome (SJS) is a disease that causes skin exfoliative lesions, characterized by fever, necrosis, and epidermal detachment. Biological skin substitutes may be considered interesting options for the treatment of this disease. This study aims at identifying in the literature the evidence on the current role of these biomaterials in the treatment of SJS and its related diseases. A systematic review with a search period between 2003 and 2017 was carried out, consulting the Lilacs, BVS, and PubMed databases. The quality of the included studies was evaluated by the Oxford Center for Evidence-Based Medicine Classification, for evaluating levels of evidence from the scientific publications. Ninety-five publications were found, and after applying inclusion and exclusion criteria, they resulted in 9 articles. In total, 20 patients with 73.87% average of body surface affected received SJS skin treatment with some biological substitutes, among them are cutaneous allograft, amnion, and xenograft. Mortality rate was 10%, and in these situations, literature indicates mortality rates ranging from 25% to 70%. Effectiveness of the use of biological dressings may be a possible explanation for this finding. Findings indicate the use of these biomaterials may favor reepithelialization, reduce water loss, decrease the chance of infection, and, consequently, improve the survival of patients with SJS and toxic epidermal necrolysis. Biological skin substitutes have characteristics that make them very promising in the topical treatment of these wounds, but their use remains very restricted in the treatment of SJS.

English

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are adverse drug reactions. They occur in the form of mild and sometimes severe cutaneous eruptions, with high morbidity and mortality, requiring fast and appropriate diagnosis and treatment. The aim of this study was to describe and discuss the evidence regarding the risk factors, complications and treatment of SJS and TEN in inpatients. The present study is a literature review of case reports published between January 1981 and December 2016, in the following databases: The Virtual Health Library (Biblioteca Virtual da Saude - BVS), MEDLINE (National Library of Medicine, USA), LILACS (Latin American and Caribbean Health Sciences Literature), and PUBMED – NCBI (National Center for Biotechnology Information). A total of thirteen case reports were selected. Most of the cases (54%) developed adverse reactions to anticonvulsant drugs. The most common were valproic acid, lamotrigine and carbamazepine. In 69% of all cases, the patients were female. The patients’ age range varied from 18 to 82 years old. In two cases which correspond to 15%, the patients were either infected with HIV or were receiving chemotherapy treatment for cancer. These patients had a higher possibility for immunosuppression. In relation to the actions taken as treatment, the suspension of the drugs or the treatment of the skin lesions occurred in 85 and 54% of the cases, respectively. A total of 6 cases, corresponding to 46%, occurred in the Latin American and Caribbean region. In conclusion, the use of anticonvulsants, and female gender, are among the main risk factors identified by the study. The main therapeutic action for SJS and TEN is the suspension of the use of the drug that triggered the inflammatory process and the topical treatment of the lesions caused. Key words: Stevens-Johnson syndrome, toxic epidermal necrolysis, adverse drug reaction.

Cyclosporine treatment for Stevens-Johnson syndrome/toxic epidermal necrolysis: Retrospective analysis of a cohort treated in a specialized referral center

Treatment of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) remains controversial. We sought to evaluate the impact of cyclosporine on hospital mortality in patients with SJS/TEN. All patients with SJS and TEN admitted to our center from 2011 to 2014 were treated under a standardized protocol that allowed for cyclosporine therapy if the inclusion and exclusion criteria were met. Clinical data were reviewed retrospectively. Comparative analysis was made on mortality outcomes with patients treated with cyclosporine versus what was expected based on SCORTEN. In all, 44 patients were admitted during the study period. A total of 24 patients received cyclosporine and the remaining 20 patients were treated supportively. SCORTEN predicted 7.2 deaths and 3 were observed in the group treated with cyclosporine. In the group treated supportively, SCORTEN predicted 5.9 deaths and 6 deaths were observed. The standardized mortality ratio of SJS/TEN treated with cyclosporine was 0.42 (95% confidence interval 0.09-1.22). Small sample size, retrospective design, and referral bias are limitations. The use of cyclosporine may improve mortality in SJS/TEN and needs to be validated in controlled studies.