Johnson Syndrome Research Articles

A Rare Low-Grade Glioma with Stevens Jonson’s Syndrome - Case Report

Introduction: The brain has a complicated structure within the body, but the CNS develops during growth and development in three stages: cytogenesis, histogenesis, and organogenesis, which determine the CNS's final makeup and shape. Organogenesis occurs in children when neural and extra-neural tissues mingle, causing a growing lesion inside the skull. Nearly 20% of the second-most prevalent group of paediatric neoplasms after leukaemia either a benign or malignant primary brain tumour may exist. The majority of infratentorial and close-to-midline brain tumours result in hydrocephalus. Astrocytomas, ependymomas, craniopharyngiomas, and malignant gliomas are the most prevalent supratentorial tumours. Additionally, toxic epidermal necrolysis (TEN) and Stevens Johnson's syndrome (SJS) are now thought to be variations of the same hypertension illness. These conditions affect 1 to 3 million children each year. The degree of epidermal detachment determines the classification; a transitional SJS with less than 10% and 30% epidermal detachment is most frequently confused with Kawasaki illnesses. Case presentation: 9-year-old male child came with complaints of ataxic gait in the past 7 days and vomiting in the past 20 days. As narrated by the father patient was well 7 days back then the patient had difficulty walking due to an ataxic gait, the patient fell on the ground and had a head injury and had no vomiting, fever, loss of consciousness, and no altered sensorium. The patient had 4 episodes of vomiting yesterday and 2 episodes today morning, non-bilious, non-projectile, and containing sputum, patient had a headache that was progressive, intermittent no blurring of vision, not fever but vomiting. The patient then came for further management. Conclusion: The second most prevalent type of paediatric neoplasm after leukaemia, organogenesis in the kid occurs when neural and extra neural tissue combines to form a growing lesion inside the skull either a benign or malignant primary brain tumour may exist. The majority of infratentorial and close-to-midline brain tumours result in hydrocephalus. Additionally, toxic epidermal necrolysis (TEN) and Stevens Johnson's syndrome (SJS) are now thought to be variations of the same hypertension illness. With the development of newer diagnostic methods like MRI, CT, SPECT, PET, and newer electroencephalography techniques as well as evoked potential, it is now possible to accurately diagnose and delineate a variety of neurological alignments. The incidence of these conditions ranges from 1 to 3 million per child lower the risk of morbidity.

Excessive vincristine exposure in a child being treated for acute lymphoblastic leukaemia with underlying Dubin-Johnson syndrome: a case report.

Dubin-Johnson syndrome is a rare benign autosomal recessive condition that causes an isolated increase of conjugated bilirubin in the serum. Impaired biliary excretion is due to mutation in the multiple drug-resistance protein 2 gene (MRP2). We describe the case of a 4-year-old girl being treated for acute lymphoblastic leukaemia who had a history of conjugated hyperbilirubinaemia and persistently elevated bilirubin levels on initiation of chemotherapy. During treatment for leukaemia, she was diagnosed with Dubin-Johnson syndrome for the underlying condition. Following administration of vincristine at the recommended dose of 1.5mg/m2, an abnormally high vincristine exposure was observed (AUC > 200µg/L*h), approximately 3 times higher than previously reported exposures in a comparable clinical setting. Vincristine dose reductions were applied on subsequent cycles of treatment and resulted in markedly reduced drug exposures, within the normal target range. This case provided a rare opportunity to assess the impact of MRP2 mutations associated with Dubin-Johnson syndrome on the pharmacokinetics of vincristine and strongly indicates that a marked dose reduction should be recommended. Clinicians should be made aware of the potential for altered drug disposition for agents such as vincristine in patients with this rare genetic condition.

Stevens – Johnson syndrome: a case report

Stevens – Johnson syndrome: a case report

Drug rash with eosinophilia and systemic symptoms syndrome: A prospective study at a tertiary health-care center

Abstract Purpose: Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome can be fatal if there is delay in diagnosis and discontinuation of causative drug. It is commonly linked with anticonvulsants and antibiotics. Understanding typical ocular manifestations can aid in early diagnosis and treatment. The main aim of this study is to provide an overview of common ocular manifestations of DRESS syndrome along with identifying the medications responsible and determining age and gender predilection. Methods: This is a 2-year observational study from June 2018 to June 2020. 37 patients recruited from ophthalmology OPD were thoroughly examined, and diagnosed in consultation with the microbiology and dermatology departments. Data required were collected by systematic examination of medical records. Patients diagnosed with DRESS syndrome having redness, itching, watering of eyes, diminision of vision, lid swelling, discharge, pain, and foreign body sensation were included in the study. Patients with no drug history, immediate drug hypersensitivity reactions, steven- Johnson syndrome were excluded. Results: Common ocular manifestations observed were tarsitis (14%), madarosis (12%), ulcerative blepharitis (10%) and conjunctivitis (10%), squamous blepharitis and keratoconjunctivitis (9%) along with other rare findings. Common triggering drugs were anti tubercular treatment (46%), carbamazepine (24%), dapsone (5%), antibiotics (11%). Mean age group affected was 42.05 years with peak between 11-20 years and slight female preponderance of 54%, with males being 46%. Conclusion: Knowing common ocular manifestations of DRESS syndrome and the drugs triggering is crucial in preventing serious consequences. Though treatment is supportive and steroids, key is to withdraw the causative drug early.

Diclofenac sodium and Allopurinol induced Stevens Johnson Syndrome in pulmonary tuberculosis patients receiving intensive phase first-line antituberculosis drugs

One of the life-threatening multi-organ emergency conditions is Stevens-Johnson Syndrome (SJS). Although this is a rare disease and manifests as drug reactions, this condition can cause death.We present a case of a 63-year-old male with Diclofenac sodium and Allopurinol induced Stevens_Johnson Syndrome in pulmonary tuberculosis patients receiving intensive phase first-line antituberculosis drugs.The patient was undergoing outpatient TB treatment at the end of the first month. The patient comes to the emergency department of the hospital because of shortness of breath, fever, blistered skin, nasal and oral mucosa, cracks and wounds, visible pus, red eyes accompanied by erythematous rash all over the body after previously taking diclofenac sodium and allopurinol given by the doctor because felt joint pain all over body and uric acid increased. On physical examination of the lungs, crackles and wheezing were heard in both lung fields. The chest radiograph shows infiltrates in both lung fields. Laboratory results showed leukocytosis and the results of other blood laboratory tests were still within normal limits. Patient diagnosed Stevens-Johnson Syndrome based on clinical, laboratory, and radiology examination results. The patient was given treatments using nasal canule oxygen of 5 litres/minute, intravenous fluid dehydration D5% : NaCl 0.9%, ceftriaxone injection, gentamicin injection, dexamethasone injection, cetirizine ranitidine injection, compresses with 0.9% NaCl liquid, 2.5% hydrocortisone ointment. The patient was treated in the Intensive Care Unit for 8 days. The patient had no history of previous drug allergies. In this case, the likelihood of Diclofenac sodium and Allopurinol induced Stevens_Johnson Syndrome in pulmonary tuberculosis patients receiving intensive phase first-line antituberculosis drugs, needs to be a concerned, as well as the importance of evaluation and strict follow-up to prevent Stevens Johnson Syndrome disease.

CD12 Citalopram-induced drug reaction with eosinophilia and systemic reaction confirmed by patch testing

Abstract A 46-year-old man was referred for investigation of an acute widespread rash and fever that started 4 weeks after commencing citalopram. Clinical and histological features were consistent with drug reaction with eosinophilia and systemic reaction (DRESS). He was not on any other medication, had no known allergies and had not previously taken a selective serotonin reuptake inhibitor (SSRI). Six months after resolution, he was patch tested to the extended British Baseline Series and citalopram (crushed tablet in 50% petrolatum). Readings on days 2 (D2) and 4 (D4) were positive for citalopram (D2 ++ and D4 +++). To investigate possible crossreactivity, he was subsequently patch tested to fluoxetine, paroxetine and sertraline (crushed tablets in 50% petrolatum), which showed a positive reaction to paroxetine [D2 (–) and D4 (+)]. Patch tests to citalopram and paroxetine were negative with no irritancy in a normal volunteer. SSRIs are extensively and increasingly used for depression and other mental health problems; 45 000 000 UK prescriptions were issued in the last financial year. Despite such widespread use, cutaneous adverse drug reactions (CADRs) appear to be rare. From 1998 to 2022, the Medicines and Healthcare products Regulatory Agency recorded 16 586 adverse reactions to citalopram, of which only 1077 (6.5%) were cutaneous. Various rashes have been reported to arise from SSRI treatment, ranging from maculopapular eruptions to toxic epidermolysis necrolysis/Stevens–Johnson syndrome. There have been isolated reports of DRESS from fluoxetine, sertraline and escitalopram (the S-enantiomer of citalopram). To our knowledge, this is the first reported case of citalopram-induced DRESS. The sensitivity and specificity of patch tests in the diagnosis of delayed CADR varies according to the drug and rash. We have found case reports of positive patch tests to sertraline and paroxetine following maculopapular exanthems. Our findings highlight a diagnostic role for patch tests with an extemporaneous preparation in SSRI-induced DRESS. SSRIs have diverse chemical structures, and we speculate that patch tests may be of benefit in identifying a safer alternative should further SSRI treatment be needed.

P103 A retrospective observational study of toxic epidermal necrolysis at a tertiary referral centre from 2015 to 2022

Abstract Toxic epidermal necrolysis (TEN) is associated with high mortality and morbidity. Owing to a lack of robust evidence, treatment guidelines are ambiguous and clinicians must use discretion in management decisions, specifically with regard to the use and choice of systemic agents, including immunosuppressants. We present a retrospective study of cases referred to a tertiary referral centre between 2015 and 2022. Cases were identified using histopathology coding and a database compiled by clinicians. Forty cases were categorized as follows: TEN (n = 12); Stevens–Johnson syndrome (SJS)/TEN overlap (n = 5); SJS (n = 5); unclear (n = 9); insufficient information (n = 5) and others [n = 9 (lupus, n = 4; erythema multiforme major, n = 3; burn, n = 1; recurrent erythema multiforme, n = 1)]. The median age of patients with TEN was 66 [interquartile range (IQR) 53.3–74; 33% men, 67% women]. The median percentage body surface area of epidermal loss was 40% (n = 8; IQR 30–70). Forty-two per cent of patients had an underlying malignancy. Implicated medications included nivolumab/ipilimumab (n = 2), co-trimoxazole (n = 2), carbamazepine (n = 1) and proton pump inhibitors (n = 3). Sixty-seven per cent of patients with TEN were managed by the burns centre, with a further 17% admitted to the intensive care unit. One patient presented directly to the dermatology team and 58% were transferred from other hospitals. The median time from the documented onset of rash to transfer to specialist care was 5 days (IQR 2.8–11.5). Thirty-three per cent of patients were given immunosuppression (intravenous methylprednisolone). The median duration of admission was 14 days (IQR 7.5–21.5). Fifty-eight per cent of patients (n = 7) survived. None of the five patients who died had received immunosuppression. The median time from onset of rash to receiving specialist care was 3.5 days for those who survived (n = 4) vs. 10 days for those who did not (n = 3; P = 0.03). Thirty-three per cent of patients had documented chronic complications (severe ophthalmic sequelae, n = 2; circumcision for phimosis, n = 1). This study was limited by the retrospective collection of data and the method used to identify cases. The current evidence for the treatment of TEN is primarily derived from small case series that often combine heterogeneous cases of TEN, SJS/TEN overlap and SJS. This is highly problematic as the risk–benefit dynamic varies greatly between patients with SJS, who have an excellent prognosis without treatment, and patients with TEN with a high associated mortality, who potentially have more to gain from immunosuppression. Our data support the need for patients to access specialist care promptly, to allow for early consideration of immunosuppression. Future research should focus on effective treatments for reducing mortality in cases of TEN specifically.

002 National clinical audit on the management of Stevens–Johnson syndrome/toxic epidermal necrolysis in adults

Abstract UK guidelines for the management of Stevens–Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) in adults, published by the British Association of Dermatologists (BAD) in 2016, outline a set of audit standards (Creamer D, Walsh SA, Dziewulski P et al. U.K. guidelines for the management of Stevens–Johnson syndrome/toxic epidermal necrolysis in adults 2016. Br J Dermatol 2016; 174:1194–227). Our aim was to audit the current management of SJS/TEN in adults against audit standards in the BAD guidelines. BAD members were invited to submit data on five consecutive adults with SJS/TEN per department over an 8-week period in 2022. All data were obtained from respondents using a Microsoft Excel spreadsheet proforma returned to the BAD by email. No patient-identifiable data were requested. Initial data extraction was conducted using a novel and reproducible program written in the R statistical language (V3.7 and the readxl, ggplot2 and ggthemes packages). The data analysis was also performed using Microsoft Excel. Thirty-nine (29%) dermatology centres in the UK participated and data for 147 adults with SJS/TEN were collected. Within 24 h of a diagnosis being made or suspected, the following were documented: Severity-of-Illness Score for TEN (SCORTEN) for 52% (n = 76/147) of submitted cases, list of medications for 77% (n = 113) and timelines for commencement/alterations of medications for 71% (n = 104). Initial assessment was of the eyes (by an ophthalmologist) for 48% (n = 71) of cases, the mouth in 88% (n = 130), the genital skin in 70% (n = 103) and the urinary tract in 63% (n = 93). In the first 10 days after a suspected or confirmed diagnosis of SJS/TEN, daily assessments of the mouth were documented in 18% (n = 26) of cases, the eyes in 8% (n = 12) and the urinary tract and genital skin in 10% (n = 14). Documentation regarding advice on (i) avoidance of the culprit drug was present for 58% (n = 76/130) of cases and (ii) request for a medic alert bracelet/amulet in 6% (n = 9/147). In adults with SJS/TEN, drug causality assessment was documented adequately. Initial assessment of the mouth, eyes, urinary tract and genital skin was good to fair; however, daily assessments were poor. Other audit standards that require improving include documentation of SCORTEN and advice to avoid the culprit drug and request a medic alert bracelet/amulet. In adults with SJS/TEN, there are multiple points in the assessment pathway that require careful documentation and regular review. These are a challenge for many clinical inpatient environments. Care within a burns unit or similar may not be possible for all. This audit suggests that some form of assessment and clinical review checklist might enable colleagues to maintain standards outlined in the guideline. Among these, use of SCORTEN, advice to avoid the culprit drug and requests for a Medic Alert bracelet/amulet are key.

Lip Synechia: An Unusual Complication in Stevens–Johnson Syndrome Amenable to Surgical Correction

Lip Synechia: An Unusual Complication in Stevens–Johnson Syndrome Amenable to Surgical Correction

Anti-Monkeypox Infection Approaches: From Prevention to Therapeutic Lines.

Anti-Monkeypox Infection Approaches: From Prevention to Therapeutic Lines.

Adverse cutaneous reactions to psychotropic drugs

IntroductionPsychotropic drugs are not devoid of side effects, especially cutaneous reactions, some of which are severe, and can jeopardize functional and life prognosis. The Aim of this study is to highlight the epidemiological and clinical features of severe cutaneous adverse reactions (SCARs) due to psychotropic drugs. Materials and methodsWe carried out a retrospective study over a period of 10years, It concerned all patients hospitalized in our dermatology department for SCARs induced by psychotropic drugs. The imputability was confirmed by the French methodology of Bégaud et al. ResultsWe identified 41 cases, of which 18 patients (43.9%) were being treated with psychotropic drugs for the first time. The most incriminated psychotropic drugs were: carbamazepine (n=16), phenobarbital (n=8), lamotrigine (n=5). Clinical forms were: drug reaction with eosinophilia and systemic symptoms syndrome (n=30), Stevens–Johnson syndrome (n=4), overlap syndrome (n=1), drug-induced vasculitis (n=4), symmetrical drug-related intertriginous and flexural exanthema syndrome (n=1), photosensitivity (n=1). Complications were as follows: hepatic cytolysis (75%), cholestasis (60%), functional renal failure (43%). Carbamazepine was statistically correlated with renal damage and cholestasis (P=0.013) and phenobarbital was associated with a risk of hepatic cytolysis (P=0.027). ConclusionCutaneous side effects to psychotropic drugs remain rare<2%. In our study, we found that rapid or recent increase in dose of anticonvulsants, or the combination of several psychotropic drugs is the most common cause of SCARs. We also found that carbamazepine was more likely to cause renal damage and cholestasis, whereas chlorpromazine was statistically correlated with hepatic cytolysis.

Steven Johnsons Syndrome Induced by Carbamezapene

Steven Johnsons Syndrome Induced by Carbamezapene

Steven Johnsons Syndrome Induced by Carbamezapene

Steven Johnsons Syndrome Induced by Carbamezapene

Dapsone hypersensitivity syndrome.

Dapsone hypersensitivity syndrome.

004 Clinicopathological and molecular profile of Stevens–Johnson syndrome and toxic epidermal necrolysis-like acute graft-versus-host disease

004 Clinicopathological and molecular profile of Stevens–Johnson syndrome and toxic epidermal necrolysis-like acute graft-versus-host disease

Allopurinol hypersensitivity syndrome.

Allopurinol hypersensitivity syndrome includes Stevens–Johnson syndrome, toxic epidermal necrolysis and drug reaction with eosinophilia and systemic symptoms. It affects about 1 in 1000 patients prescribed allopurinol, and reported mortality is between 20% and 25%.[1][1] Patients typically present

5-Fluorouracil as a targeted lacrimal gland therapy for chronic Stevens-Johnson syndrome: A pilot study.

The surgical technique of periglandular 5-fluorouracil (5-FU) injection and its effects on the morphology and function of the main lacrimal gland of patients with severe dry eye disease due to Stevens-Johnson syndrome (SJS) are reported. 5-FU, as a potential antifibrotic agent, is given in the dose of 0.1 ml (50 mg/ml), subconjunctivally into the periglandular fibrosed area of the palpebral lobe of the main lacrimal gland. The injection is given using 30G needle into the subconjunctival plane and not into the substance of palpebral lobe. Eight eyes (eight lobes) of seven chronic SJS patients (mean age, 32.5 years, <5 mm Schirmer) received the injection. All eight lobes demonstrated a visible reduction in the conjunctival congestion and scarring over the lobar area. The mean OSDI scoring improved from 65.3 to 51.1. Three patients with mean pre-injection Schirmer I values of 4 mm showed a mean change of 1 mm at four weeks following a single injection. The tear flow rate per lobe for the above three patients improved from 0.22, 0.12, and 0.16 μl/min to 0.31, 0.12, and 0.21 μl/min, respectively. Another patient with pre-injection Schirmer of 4 mm showed no change in tear flow. Three eyes with zero baseline Schirmer values (no visible secretory opening) had no improvement in tearing or ocular surface staining. Local 5-FU injection alters morphology of the conjunctiva overlying the palpebral lobe in SJS patients, but fails to show any significant effect on tear secretion.

Stevens - Johnson syndrome After Anti-Tubercular Therapy in a Patient with Tuberculosis of the Spine

This case is of a 54-year-old female showing a rare and life-threatening steven johnson syndrome (Toxic epidermal necrolysis) as an adverse reaction after starting with Anti-tubercular therapy for tuberculosis of the spine (D8-D9). The patient developed a cutaneous reaction 10 days after starting treatment for Tuberculosis. The documentation of Steven Johnson syndrome after starting anti-tubercular drugs is sparse and Tuberculosis is a high-incidence and prevalence disease, the drugs used to treat the same are widely used.

Personalized 3D-printed amniotic fornical ring for ocular surface reconstruction.

In the present work, we used three-dimensional (3D) printing technology to make a polylactic acid (PLA) amniotic fornical ring (AFR) for ocular surface reconstruction. This work is a retrospective and interventional case series of patients with ocular surface diseases who underwent either personalized 3D-printed AFR-assisted amniotic membrane transplantation (AMT) or sutured AMT (SAMT). Patient epidemiology, treatment, operative duration, epithelial healing time, retention time, vision changes, morbidity, and costs were analyzed. Thirty-one patients (40 eyes) and 19 patients (22 eyes) were enrolled in the 3D-printed AFR group and the SAMT group, respectively. The clinical indications of AFR and SAMT were similar, such as corneal and/or conjunctival epithelial defects due to chemical burns, thermal burns, Stevens-Johnson syndrome (SJS), or toxic epidermal necrolysis (TEN). The mean dissolution time was 15 ± 11 days in the AFR group, compared with 14 ± 7 days in the SAMT group. The percentage of healed corneal area was 90.91% (66.10%-100.00%) for AFR and 93.67% (60.23%-100.00%) for SAMT. The median time for corneal epithelial healing was 14 (7-75) days in the AFR group and 30 (14-55) days in the suture AMT group. There were no significant differences in the initial visual acuity, final visual acuity, or improvement in visual acuity between the two groups. The operation duration in the AFR group was significantly shorter than that in the SAMT group. Regarding the cost analysis, the average cost per eye in the AFR group was significantly lower than that in the SAMT group. Furthermore, 3D-printed and sterile AFR showed no obvious side effects on the eyes. Our results suggested that 3D-printed PLA scaffolds could be used as an AFR device for ocular surface disease. In addition, personalized 3D-printed AFR is superior to conventional AMT in operation duration and cost effectiveness, thereby reducing the financial burden on our health care system.

Vaccines for monkeypox disease and challenges in its production and distribution: a lesson from COVID-19 pandemic.

Vaccines for monkeypox disease and challenges in its production and distribution: a lesson from COVID-19 pandemic.