Role of amniotic membrane transplantation in treating acute ocular manifestations in Steven–Johnson syndrome and its long-term impact

Background: Steven–Johnson syndrome (SJS) is a serious disorder affecting the skin and mucous membrane, causing multiple flaccid bullae and purpuric rashes with sheet?likeepithelial detachment including the ocular surface. The long?term outcomes following SJS are dismal and manifest as corneal vascularization, lid?wiper keratopathy, and severe dry eyes. The disease course can be modified if amniotic membrane graft is performed at the first week of disease, and the above?said complications can be avoided. This procedure thus not only decreases the morbidity but also improves the quality of life. Purpose: This video discusses the long?term sequelae of SJS which can be modified with timely intervention during the acute stage and thus significantly decreases morbidity. Synopsis: The video demonstrates the simple technique of amniotic membrane transplantation in the acute stage in SJS and its impact in long run. Highlights: There should be a low threshold for doing early amniotic membrane transplantation in patients with SJS with ocular surface involvement. Early intervention can change the disease course and decrease disease morbidity significantly.

Systemic lupus erythematosus patient on steroid therapy that develop to a Stevens-Johnson syndrome: a case report

Stevens-Johnson syndrome (SJS) is a cutaneous immunity reaction involving the skin and mucosa and is an emergency condition that can be fatal. The incidence of this disease is relatively rare in the range of 1-2 per 1.000.000 population. The pathogenesis of SJS involves the immune system response ofantigenic drug to body tissuesbut it still cannot be fully explained to date.We reported a woman, 35 years old with systemic lupus erythematosus (SLE) who had been on steroid therapy and in the course of treatment developed into SJSafter administration of anti-epileptic drug. Steroidshaveanti-inflammatory effects mainly due to decreased syntheses or suppression of inflammatory mediators.SJSstill can develop in patient with SLE who had been on steroid therapy. Giving steroid that indicated for the treatment of a disease including SLE, cannot prevent the occurrence of an allergic event including SJS.The presence of steroid can extend the duration of starting the drug with the occurrence of SJS and reduce the severity of the disease. Steroid still have a role in treatment that can be used both in SJS and SLE.

ALLOPURINOL INDUCED ADVERSE DRUG REACTIONS: CASE SERIES

Allopurinol is widely used drug for patients of hyperuricemia and gout. The few adverse drug reactions related to allopurinol are diarrhea, fever, hepatotoxicity, Steven Johnson Syndrome- Toxic Epidermal Necrolysis (SJS-TEN), congestive heart failure and renal failure. In this article we have described eleven cases to evaluate the causality, cause and management of this syndrome. In this study eleven retrospective cases were selected from the ADR reports. The full details of the cases were collected from Christian Medical College from the period of 2012 to 2016. Causality was established using Naranjo scale and classi?cation was done based on severity and resolution of ADR. It was observed that cases presented with mild to severe reactions of allopurinol, which is the suspected offending agent. Cases were resolved with supportive treatment while ?ve case had a prolong hospitalization and two cases had a life threatening drug reaction. Early detection and management of this severe form of systemic reaction is very important for better will be the prognosis.

Stevens-Johnson syndrome and toxic epidermal necrolysis: a review

Stevens Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are considered a single entity with variability in the extent of the lesions, characterized by erythema multiforme that may involve mucosa. Severe cutaneous reactions secondary to medications are classified according to the area of epidermal detachment. The activation of cytotoxic T cells and macrophages is mediated mainly by IL-2 and interferon gamma secreted by Th1 lymphocytes, and the activation of eosinophils and B lymphocytes in IgE is mediated by secreted IL-4, IL-5, IL-10 and IL13 by B lymphocytes. The topography of SJS is predominantly central, affecting the trunk and sometimes a generalized dissemination is shown that affects a body surface area of less than 10%, characterized by irregular violaceous erythematous macules of target shooting, which can form confluent blisters. TEN is characterized by a skin detachment greater than 30% of the body surface, whose predominant lesion is diffuse erythema with individual macules, which give rise to detachment surfaces greater than 5 cm. The treatment is symptomatic, nonspecific, and aimed at avoiding complications, carried out in specialized intensive care units, due to ignorance of the pathogenesis. Integral management with different therapeutic alternatives can represent a crucial part in the multisystemic management of SJS and TEN.

An observational study to monitor cutaneous adverse drug reaction profile in Dermatology Department of tertiary care teaching hospital

Background: Incidence of cutaneous adverse drug reactions (CADRs) in developed countries is 1 to 3% and in developing countries, it is much higher i.e. 2 to 6%. 1 in 1000 hospitalized patients will develop severe cutaneous adverse reaction. Maculopapular rash represents majority of cutaneous drug reaction followed by urticaria. Most frequently elicited CADRs are associated with antimicrobials and NSAIDs. This study was designed to monitor Cutaneous adverse drug reaction profile of tertiary care teaching hospital.Methods: This is a prospective observational study of 6 months� duration to monitor cutaneous adverse drug reactions in dermatology department of tertiary care teaching hospital. CADRs were analysed with respect to demographic details, suspected drugs and type of reaction. Causality assessment is by Naranjo algorithm. Data is represented in tables and graphs. Data is analyzed in Microsoft excel 2007.Results: Total 57 cases of cutaneous adverse drug reactions were reported. Among them, 57.9% were in males and 42.1% were in females. Majority of CADRs were due to antiretroviral drugs (38.5%) followed by antibacterial (28%) and antiepileptics (14%). Maculopapular rash is most common CADR (35%). Causality of 74% CADRs were probable according to Naranjo algorithm.Conclusions: CADRs are more commonly associated with antiretroviral therapy (ART), antibacterial drugs and antiepileptic drugs. In case of ART, antiepileptic drug and drugs used in chronic illness compliance plays a major role in the success of therapy. Adverse drug reactions lead to problem of non compliance and failure of therapy. Cutaneous adverse reactions like FDE heal with hyper pigmentation leads to cosmetic problem. Stevens Johnson syndrome (SJS) is life threatening that requires prompt withdrawal of drug and intensive medical management. Many drugs are available without prescription in India leading to problem of misdiagnosis of CADRs. So, data obtained from this study helps in proper diagnosis and treatment of CADRs.

Phenytoin induced Stevens Johnson syndrome: a case report

Stevens-Johnson syndrome (SJS) is a rare consequence of hypersensitivity reaction precipitated by certain drugs and viral infections. It is an idiosyncratic drug reaction usually associated with drugs like anti-epileptics, non-steroidal anti-inflammatory compounds and antibiotics. The overall incidence of this entity is very low and is life-threatening if undiagnosed and untreated. The syndrome is characterized by purpuric macules and bullous eruptions involving the mucous membrane which may be followed by systemic manifestations. The mechanism of SJS due to drugs is not fully defined. Delayed Hypersensitivity reaction mediated by T lymphocytes in response to a drug is thought to be responsible. Here authors present a case of SJS induced by phenytoin in an adult male. The case warrants the need of adopting a meticulous approach while prescribing phenytoin. The case is being reported to accentuate the importance of adverse drug reactions and to emphasize the importance of reporting such reactions ensuring efficient pharmacovigilance.

Phenytoin induced Stevens-Johnson syndrome: a case report

Toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS) are rare but potentially life threatening cutaneous adverse drug reactions. Drugs commonly implicated are anti-microbials, anti-epileptics and non-steroidal anti-inflammatory drugs (NSAIDs). Amongst anti-epileptics, carbamazepine and phenytoin are the most common offending drugs. We report here a case of SJS due to phenytoin.

An Epidemiological Study of Stevens-Johnson Syndrome (SJS), Toxic Epidermal Necrolysis (TEN) and Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN) overlap in University Malaya Medical Centre

Introduction: Steven-Johnson syndrome and Toxic Epidermal Necrolysis are rare but life threatening severe cutaneous adverse reactions to drugs. To determine the epidemiology of SJS, TEN and SJS/TEN overlap in University Malaya Medical Centre (UMMC). Methods: All patients admitted to UMMC from year 2013-2015 for SJS, SJS/TEN, TEN were recruited. The classification of SJS, SJS/TEN overlap and TEN was made based on the criteria laid down by Bastuji et al.2 Results: A total of 32 patients were recorded to have SJS, SJS/TEN overlap and TEN from 2013 to 2015. Drugs (n=32, 86.49%) remained the most common aetiology of SJS and TEN. The top three commonest drugs are allopurinol (n=6), followed by carbamazepine (n=5) and bactrim (n=3). Conclusion: This study demonstrates that drugs were the most common cause of SJS/TEN. Antibiotics were the most common drug group that caused SJS/TEN. Awareness of the common etiology such as drug is important and high index of suspicion of SJS and TEN is needed if patients were on the above medications.

Toxic Epidermal Necrolysis- A Caution before Prescribing Anti-Epileptics.

We report a case of Toxic Epidermal Necrolysis (TEN) in a 10 year old male child resulted from inadvertent anti-epileptic usage. Steven-Johnson Syndrome (SJS) /TEN has been seen as a side-effect of many drugs like Cotrimoxazole, Phenytoin, Carbamazepine, Valproic acid, NSAID’S, Allopurinol. Etc., but TEN in a patient who is on valproic acid with addition of lamotrigine is rare which is a point of interest in our case which was successfully treated with Intravenous immunoglobulins.

A 5 Year Retrospective Study on the Clinical Patterns and Treatment Outcomes of Severe Cutaneous Adverse Drug Reactions (SCARS) in Hospital Tengku Ampuan Rahimah, Malaysia

Introduction: Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN) , and drug reaction with eosinophilia and systemic symptoms (DRESS) are severe cutaneous adverse drug reactions (SCARs) related to a variety of medications. Objectives: We aim to document the epidemiological features, the causative drugs and clinical outcomes of patients with SCARs treated in Hospital Tengku Ampuan Rahimah (HTAR) between January 2009 and December 2013. Materials & Methods: A retrospective review of the data of all patients with SJS, TEN and DRESS treated from January 2009 to December 2013 was retrieved and analyzed. Results: A total of 33 SCARs patients were seen, which included SJS (25), TEN (3) and DRESS (5). The mean age was 42.8 years. The male-to-female ratio was 1.36:1. Allopurinol (33.3%) was the commonest offending drug, followed by antibiotics (30.3%), anticonvulsants (12.1%), non-steroidal anti-inflammatory drugs (12.1%) and traditional medications (6.1%). Eighty percent of SJS and all TEN and DRESS patients were given systemic corticosteroids. One patient with TEN (33.3%) was concurrently given intravenous immunoglobulin. All SJS patients survived. Two patients with TEN (66.7%) and one patient with DRESS (20%) succumbed due to sepsis. Conclusion: The commonest drugs implicated for SCARs in our study were allopurinol and antibiotics. Inappropriate use of these drugs leads to increased risk of SCARs. Early recognition and prompt treatment of patients with SCARs may improve their outcome.

Pharmacogenetic research in the association between human leukocyte antigen and adverse drug reactions / 中南大学学报(医学版)

With the rapid development of pharmacogenetics, more and more studies have shown evidence in the association between polymorphisms at the human leukocyte antigen (HLA) loci and severe adverse drug reactions (SADRs). Several HLA-B alleles proved to be associated with SADRs for drugs such as carbamazepine, allopurinol, lamotrigine, and lfucloxacillin. hTe USA Food and Drug Administration (FDA) has even recommended routine screening for HLA-B allele before the use of abacavir and carbamazepine. With the completion of human genome project and the Hapmapproject, several new pharmacogenetics approaches such as genome-wide association study (GWAS) have emerged. hTese newly developed methods will undoubtedly accelerate the identiifcation and clinical utilization of the pharmacogenetic biomakers. In addition, the immunogenetic mechanisms by which the HLA alleles cause SADRs are explored at the cellular and molecular level. hTis review focuses on the recent progresses in HLA alleles and ADRs regarding both the clinical translation and modern pharmacogenetic methods.

<b>4. Safety Risk Evaluation Methodology in Detecting the Medicine Concomitant Use Risk which might Cause Critical Drug Rash</b> / 薬剤疫学

The new practical use example of the JADER datasets from Japanese Adverse Drug Event Report database opened by Independent Administrative Agency Pharmaceuticals and Medical Devices Agency in April, 2012 and afterwards will be reported. The purpose of this study is to examine the evaluating method of medicine concomitant use risk by the frequency at which two or more medicines were reported simultaneously, being assumed the possibility of the influence of drug interactions to be the concomitant use risk in an adverse drug event onset. In order to estimate the potential degree of the safety risk at the time of the concomitant use, the methodology was estimated by the following procedures. 1) For considering that two suspicion medicine targeted is one medicine, the statistical signal index which means those of medicines with use in the case where they both are indicated in one report, the index of the concomitant use, is computed. 2) The statistical signal index about two target suspicion medicines is computed individually. 3) The case where the ratio of the index of the concomitant use to the index obtained individually exceeds 2 also in any of two suspicion medicines is judged as there being the concomitant use risk. The Proportional Reporting Ratio (PRR) and the Reporting Odds Ratio (ROR) were used as a statistical signal index. In order to check the validity of this method, Stevens-Jonson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) which are known for the adverse events of critical drug rash reported in JADER were taken up, and the causality of medical supplies limited to the medicine with which they were reported as a suspicion medicine. As for the combination of the suspicion medicine which fulfills the conditions of the concomitant use risk, 10 kinds of candidates out of 159 combinations for SJS and 22 kinds of candidates out of 111 combinations for TEN were detected, respectively. Although this approach for the concomitant use risk was considered to be an effective means in showing the above results, some issues about the ratio of the index of the concomitant use and criteria in the report numbers of the medicine to be chosen, the effective calculation method for combinations in more than 3 medicines, etc. will be required for the further examination.

Fatal Stevens-Johnson syndrome induced by phenytoin: a case report.

Toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS) are rare (one to two per 10,00,00 population per year) but life threatening adverse drug reactions. Drugs commonly implicated are anti-epileptics, anti-microbials and non-steroidal anti-inflammatory drugs (NSAIDS). Amongst anti-epileptics, carbamazepine and phenytoin are the major culprits. We report here a fatal case of SJS due to phenytoin.

Steven Johnson Syndrome / Síndrome de Steven Johnson

Ulcerative vesiculobullous disorders are common in Dermatology and Oral Medicine. Diagnosis of these conditions is pretty effortless if they give a classic appearance as described in the literature. Steven Johnson syndrome is one such condition which is a type of erythema multiforme. It is a disorder involving mucous membrane, skin and even the multiple organs in severe form. Multiple etiologies persist so be acquainted with the cause and prohibit the root is crucial. But still drugs are considered to be common cause. Early management is vital as complications are serious for this condition even leading to death. We hereby report a typical case with classic appearance of Steven Johnson syndrome.

Doenças ulcerativas vésicobolhosas são comuns em Dermatologia e Medicina Oral. O diagnóstico destas condições é bastante fácil se apresentar uma aparência clássica, tal como descrito na literatura. Steven Johnson é um tipo de eritema multiforme. É uma desordem envolvendo as mucosas, pele e até múltiplos órgãos de forma grave. Múltiplas etiologias são apontadas como causa. Mas ainda os medicamentos são considerados como causas comuns. O tratamento precoce é vital, pois complicações são graves para essa condição, podendo levar à morte. Vimos por meio deste relatar um caso típico, com aparência clássica de Síndrome de Steven Johnson.

A 4-year retrospective study of Stevens-Johnson syndrome and toxic epidermal necrolysis

Background Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare bullous mucocutaneous disease usually caused by drugs. We aim to determine the demographics, causes and outcomes of patients admitted with SJS, TEN and SJS-TEN overlap in Sarawak General Hospital. Materials and Methods A retrospective review of cases admitted to Sarawak General Hospital with SJS, TEN and SJS-TEN overlap from January 2004 to December 2007 was undertaken. Data regarding the demographic, causes and outcomes were collected from the case folders and subjected to descriptive statistical analysis using Microsoft Excel. Results Twenty four cases were admitted with 54.2% having SJS, 25% having SJS-TEN Overlap and 20.8% having TEN. With the mean ages of more than 40 years, patients with SJS and SJS-TEN overlap were older than patients with TEN, with a mean age of only 25.4 years. Seventy nine percent of cases were drugs induced. Anticonvulsants were the main culprit constituting 29.2% followed by allopurinol with 20.8%. Cases with SJS had the longest incubation period with mean of 21.6 days whereas cases with TEN had the longest mean hospital stay with 12.4 days. A 12.5% mortality rate was recorded with 2 deaths in the SJS-TEN overlap group and one death in the TEN group. All cases who were given intravenous immunoglobulin (IVIg) survived. Conclusion SSJS, SJS-TEN Overlap and TEN were mainly drug induced and have high mortality. IVIg treatment seems promising. Early recognition and optimal care in institution with dermatology service is essential in reducing morbidities and mortalities.