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Background: Sulfonamides are associated with severe cutaneous adverse reactions (SCARs). Coronavirus disease 2019 (COVID-19) triggers an immune response, which may increase the likelihood of developing a hypersensitivity reaction. Objectives: We sought to explore the impact of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection on the probability of developing SCARs and/or erythema multiforme (EM) reactions to sulfonamides. Methods: In the propensity score-matched cohort study by using the de-identified TriNetX Research data base, patients who had an exposure to antibiotic or non-antibiotic sulfonamides between March 1, 2020, and January 1, 2023, were divided into two groups based on the presence or absence of a previous COVID-19 infection within 6 months of starting the sulfonamide agent. The outcomes studied were the 30-day risk of developing SCARs or EM (Stevens-Johnson syndrome, toxic epidermal necrolysis, drug reaction with eosinophilia and systemic symptoms, or EM) within 3 months of sulfonamide exposure. Cohorts were matched based on baseline demographics; malignant lymphoid neoplasms; human immunodeficiency virus; systemic lupus erythematosus; bone marrow transplantation; diabetes; psoriasis; seizures; gout; solid organ or stem cell transplantation; COVID-19 vaccination; and exposure to risk medications, including allopurinol, levetiracetam, carbamazepine, lamotrigine, oxcarbazepine, phenytoin, phenobarbital, abacavir, nevirapine, piroxicam, tenoxicam, or mexiletine. Results: When comparing 345,119 patients on sulfonamides and with previous COVID-19 to an equal number of sulfonamides users without a previous COVID-19, patients with COVID-19 had a lower risk of developing any form of SCARs (relative risk 0.39 [95% confidence interval, 0.26, 0.58]; p < 0.001). Conclusion: Previous SARS-CoV-2 infection seems to be associated with a lower probability of developing SCARs or EM among patients using sulfonamides. [ABSTRACT FROM AUTHOR]

Purpose: To report a challenging case of corneal descemetocele following Nd:YAG laser capsulotomy for posterior capsule opacification in a patient with Steven Johnson syndrome (SJS). Observations: A single-eye 52 year-old man, with a history of Steven Johnson syndrome, presented with severe eye pain and profound vision reduction in his left eye two days after undergoing Nd:YAG laser capsulotomy using the standard Abraham contact lens. A corneal descemetocele was identified and subsequently confirmed by anterior segment optical coherence tomography. He was promptly treated with the application of a therapeutic contact lens and sustained antibiotic regimen (preservative-free fluoroquinolone drops every 4 hours for 6 weeks) until healing of the corneal epithelium. Throughout the following eight weeks AS-OCT showed favorable anatomical and functional outcomes, achieving a substantial spontaneous healing. Conclusions and Importance: Corneal descemetocele may occur after Nd:YAG laser capsulotomy in patients with Steven Johnson syndrome. This case strengthens the critical importance of a careful preoperative assessment and meticulous postoperative management in high-risk patients, such as those with Steven Johnson syndrome, even after seemingly routine and safe ophthalmic procedures.

Key Clinical Message: Toxic Epidermal Necrolysis (TEN) is a rare, but potentially fatal mucocutaneous reaction, that may occur due to an immunologic response to certain medications. However, TEN triggered by Trastuzumab is extremely rare. Early diagnosis, recognition, and prompt cessation of the offending drugs and initiation of steroid therapy with supportive management are the most important actions for managing TEN. Although rare, it is important to be vigilant about this potential adverse reactions associated with trastuzumab to ensure patient safety and contribute to better outcomes. [ABSTRACT FROM AUTHOR]

Stevens Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are serious conditions that carry a high rate of morbidity and mortality. This review highlights the pearls and pitfalls of SJS/TEN, including presentation, diagnosis, and management in the emergency department (ED) based on current evidence. SJS/TEN is a rare, delayed hypersensitivity reaction resulting in de-epithelialization of the skin and mucous membranes. The majority of cases are associated with medication or infection. Clinicians should consider SJS/TEN in any patient presenting with a blistering mucocutaneous eruption. Evaluation of the skin, mucosal, pulmonary, renal, genital, and ocular systems are essential in the diagnosis of SJS/TEN, as well as in the identification of complications (e.g., sepsis). Laboratory and radiological testing cannot confirm the diagnosis in the ED setting, but they may assist in the identification of complications. ED management includes stabilization of airway and breathing, fluid resuscitation, and treatment of any superimposed infections with broad-spectrum antibiotic therapy. All patients with suspected SJS/TEN should be transferred and admitted to a center with burn surgery, critical care, dermatology, and broad specialist availability. An understanding of SJS/TEN can assist emergency clinicians in diagnosing and managing this potentially deadly disease. [ABSTRACT FROM AUTHOR]

Toxic epidermal necrolysis (TEN) is a life-threatening mucocutaneous disorder commonly caused by drugs. TEN is often treated with corticosteroids, intravenous immunoglobulin (IVIG), or cyclosporine; however, the efficacy of these treatments is controversial. Etanercept (a TNF-a antagonist) was proven to decrease skin-healing time in a randomized clinical trial. Herein, we report the case of a 44-month-old boy who developed TEN due to deflazacort as the probable culprit drug and was successfully treated with etanercept. The patient presented to the emergency department complaining of erythematous maculopapular rashes and vesicles all over the face and body, with vesicles on the hands, feet, and trunk. Symptoms started 4 days before presentation, with edema of the upper lip, which progressed to erythematous macules over the body. He was started on deflazacort for nephrotic syndrome 21 days before the visit. Approximately 20% of the body surface area (BSA) was covered by vesicular lesions. Under the diagnosis of Steven Johnson syndrome/TEN, deflazacort was discontinued, and intravenous dexamethasone (1.5 mg/kg/day), a 5-day course of IVIG (0.4 mg/kg/day), and cyclosporine (3 mg/kg/day) were administered. The lesions seemed to be stationary for 3 days, but on the 6th day of hospitalization, when IVIG was discontinued, the vesicular lesions progressed to approximately 60% of the BSA. Etanercept 0.8 mg/kg was administered subcutaneously. Lesions stopped progressing, and bullous lesions started epithelialization. However, on the 15th day, around 30% of the BSA was still involved; thus, a second dose of etanercept was administered. No acute or sub-acute complications were observed. In conclusion, the use of etanercept in children with TEN that is not controlled with conventional therapy is both effective and safe. [ABSTRACT FROM AUTHOR]

Background and Aim: The purpose of this review is to collect the oral-facial complications caused by the covid-19 vaccines and to know the reasons for their occurrence. Material and Methods: An electronic search without date restrictions was performed in the MEDLINE databases through PUBMED and google scholar. Among the 50 studies after removing duplicates, 11 articles that met the inclusion criteria were reviewed and included in the study. Result: Studies have shown that the injection of Covid-19 vaccines may cause oral side effects such as lichen planus, erythema multiforme, Steven Johnson syndrome, mucositis, and Bell's palsy. Bell's palsy (6023 cases), skin and mucosa itching (39 cases), erythema multiforme (6 cases), lichen planus (6 cases), Steven Johnson syndrome (3 cases), mucositis (1 case), angular cheilitis (1 case) ), pemphigus vulgaris (1 case) and one case with unknown cause. Pfizer vaccine is the most complicated vaccine reported in this study. Conclusion: Considering the high prevalence of covid-19 and the subsequent injection of covid-19 vaccines in order to prevent this disease, diagnosis and treatment of possible complications, especially oral and facial complications after vaccination, are of great importance. [ABSTRACT FROM AUTHOR]

Purpose: This review focuses on the optical and therapeutic benefits which can be offered to patients by evaluating them for scleral contact lenses, and the purpose of this study is to select the indications and to find out how efficient the scleral contact lenses are in treating severe corneal ectasias, highly irregular astigmatism and ocular surface diseases (OSDs) which are otherwise difficult to manage. This retrospective observational case series study analyzed a database of 50 consecutive patients (69 eyes) fitted with scleral lenses between January 2017 and December 2019. All consecutive eyes which were given scleral contact lens trials were analyzed. Results: The study population included 34 males and 16 females, with ages ranging from 7 to 80 years. The mean follow-up was 12 months. The mean wearing time in the successful wearers group was 12.2 hours per day (range 10–16). Vision improvement (optical purpose) was the reason for the fitting in 48 eyes (67.60%), and OSD management (therapeutic purpose) in 23 eyes (32.39%). In the former group, 56.52% (n = 26) had keratoconus, and 43.48% (n = 20) had irregular astigmatism other than keratoconus. Among the OSD patients, 56.52% had Steven Johnson syndrome (SJS) sequelae, 17.39% had severe dry eye, 17.39% had chemical injury sequelae, and 8.69% had xeroderma. Keratoconus patients achieved visual acuity of more than 20/32 in 92.3% of eyes. Among the SJS patients, 69.23% had more than 20/40 (38.46% had 20/20). Conclusion: There is compelling evidence in the literature that scleral contact lenses can provide good results for the challenging patient groups described in this review. We have experienced that scleral contact lenses expand the management of corneal pathologies, especially in OSDs.

Methazolamide is used to treat patients with glaucoma. However, as a sulfonamide derivative, methazolamide shares the same adverse reaction profile as other sulfa-based medications. Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare delayed-type hypersensitivity cutaneous reactions with high morbidity and mortality. Here, we report a severe SJS/TEN overlap syndrome in an 85-year-old Chinese male patient who received methazolamide 25 mg twice daily for his left eye glaucoma. The causal relationship between SJS/TEN and methazolamide was categorized as “highly likely” on the algorithm for assessing drug causality for epidermal necrolysis. In addition to the treatments with methylprednisolone and immunoglobulin, we used a special electromagnetic spectrum therapeutic apparatus to provide skin wound care. The patient had a thoroughly satisfying recovery. This is the first case report to use electromagnetic field therapy in a patient with SJS/TEN. We share our experience here and suggest that electromagnetic field therapy can provide advanced skin wound care and facilitate the recovery of SJS/TEN.

Here, we report a case of macrophage activation syndrome (MAS) following SARS-CoV-2 vaccination, which was complicated by toxic epidermal necrolysis (TEN), and provide a tabular review of similar cases of MAS and/or adult-onset Still's disease (AOSD) occurring in association with SARS-CoV-2 vaccination. Keywords: Macrophage activation syndrome; Steven Johnson syndrome; toxic epidermal necrolysis; vaccine adverse effects; SARS-CoV-2 vaccine EN Macrophage activation syndrome Steven Johnson syndrome toxic epidermal necrolysis vaccine adverse effects SARS-CoV-2 vaccine 521 524 4 02/28/23 20230401 NES 230401 Supplementary Information The online version contains supplementary material available at https://doi.org/10.1007/s10875-022-01408-0. [Extracted from the article]

Introduction: Severe Cutaneous Adverse Reactions (SCADRs) are emergency dermatologic manifestations associated with high morbidity and mortality. Their management includes immediate withdrawal of suspected causal agent followed by prompt management with drugs such as corticosteroids, cyclosporine and cyclophosphamide. Aim: To compare the effectiveness of corticosteroids alone versus cyclosporine and corticosteroids in management of SCADRs. Materials and Methods: This was a prospective observational study carried out in Indoor patients of Dermatology Department, Civil Hospital Ahmedabad, Gujarat, India, from October 2019 to September 2022. Twenty six patients were diagnosed with SCADRs and grouped according to the treatment received in two groups: corticosteroids alone (group B), and corticosteroids along with cyclosporine (group A). The efficacy was assessed based on: the days of disease arrest, days of complete reepithelialisation, duration of hospitalisation and final outcome. To know the prognosis of the patients, Score of Toxic Epidermal Necrosis (SCORTEN) score was used. Data was entered and analysed with the help of Microsoft excel ® 2019. Results: There were 14 patients in group A and 12 in group B. In a total 26 cases majority were of Stevens-Johnson Syndrome (SJS) (50%) followed by SJS-Toxic Epidermal Necrolysis (TEN) (27%) TEN (15%), Drug Reaction with Eosinophilia and Systemic Symptom (DRESS) (8%). The mean duration of disease arrest was significantly shorter in group A (n=14) when compared to group B (n=12) (p-value <0.001**). Also, the time for complete re-epithelisation was significantly shorter in group A than group B (p-value=0.025*) While no significant difference between the two groups was observed in SCORTEN score. Mortality was 3/12 in group B, nil in group A. Conclusion: Combination therapy with corticosteroids and cyclosporine leads to an early arrest of the disease progression, better prognosis and outcome in patients of SCADRs. [ABSTRACT FROM AUTHOR]

Stevens Johnson Syndrome is a rare autoimmune disorder which includes skin and mucous membrane. In India, the incidence of Stevens Johnson Syndrome (SJS) is 1.2 to 6 million patients per year. SJS is a very serious and life-threatening hypersensitivity reaction that can occurs due to infections (mycoplasma pneumonia) or as side effects of drugs (Sulfa Drugs, Phenytoin, Carbamazepine, Lamotrigine, Phenobarbital, Allopurinol, Piroxicam, Nevirapine and Diclofenac). Antibiotics can cause SJS and their contribution is around 40%. Fluoroquinolones are prescribed globally (11%) to treat lower respiratory tract infections, gastrointestinal and genitourinary infections. Norfloxacin and Ciprofloxacin are rarely associated with drug induced SJS. Most of the informative data, available on drugs induced SJS are based on case reports or case series. Here, we present three case reports of Fluoroquinolones induced SJS. In the following cases, patients had developed symptoms of SJS within two days. Whereas in Antibiotic induced SJS, it is reported that symptoms of SJS can appear within few days or even after a single dose of taking antibiotics. Therefore, we alleged that Fluoroquinolone could be the possible causative agent in our cases. The causality assessment had done based on the WHO-UMC causality scale and it was probable in all three cases of Fluoroquinolone induced SJS. This assessment generates a strong evidence that Fluoroquinolone induced SJS in all three cases. These patients were treated symptomatically with corticosteroids, parenteral solution and other non-pharmacologic agents and discharged after complete recovery.

We aimed describe the chronic ocular sequelae of Kindler syndrome. All cases of Kindler syndrome with ocular involvement that presented to a tertiary eye care center were included. Three cases of Kindler syndrome with ocular changes were reviewed. Case 1 (10 years, female) had recurrent epithelial breakdown with severe dry eye and corneal opacity secondary to keratitis. Case 2 (28 years, male) had symblepharon , ocular surface keratinization , and severe dry eye. Case 3 (16 years , female ) had partial limbal stem cell deficiency with dry eye. All cases were treated with topical lubricants, short course of low-potency steroids and immuno-modulators. Attention must be paid to the eye in addition to the oro-an-genital mucosa to avoid longterm ocular sequelae.

Stevens-Johnson syndrome (SJS) is a serious condition involving the skin and mucous membranes and is characterized by extensive necrosis and detachment of the epidermis. We present a case report of atypical SJS occurring as a complication of Mycoplasma pneumoniae infection in a young adult patient. This case report aims to add to the limited body of literature that exists on the topic and remind clinicians of the possible diagnosis of atypical SJS in the setting of mucosal rash associated with M. pneumoniae  infection., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Aleem et al.)

Purpose: To study the outcomes of minor salivary gland transplantation for severe dry eye disease secondary to chronic Steven Johnson Syndrome., Methods: It was an ambispective, interventional case series conducted at Rajendra Prasad Centre for Ophthalmic Sciences, Delhi, India from 2022 to 2023 evaluating the outcomes of minor salivary gland transplantation with anchorage of the minor salivary glands to superior rectus muscle in twenty cases of severe dry eye disease secondary to chronic Steven-Johnson Syndrome. The pre-operative clinical parameters were compared to those at post-operative 1 year follow-up., Results: At 1 year follow-up, there was an improvement in mean Schirmer-1 value (p = 0.0004), hyperemia score (p = 0.0004), keratinization score (p = 0.04), corneal epithelial defect score (p = 0.0004), corneal opacification score (p = 0.001), corneal neovascularization score (p = 0.001), palisades of Vogt score (p = 0.007), corneal keratinization score (p = 0.04) and corneal conjunctivalization score (p = 0.08)., Conclusion: The minor salivary gland transplantation is a viable management option for cases with severe dry eye disease secondary to chronic Steven Johnson Syndrome with clinical improvement in corneal and conjunctival parameters of the ocular surface., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024. Published by Elsevier Inc.)

Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are severe, life threatening immunologically mediated, mucocutaneous, Adverse Drug Reactions (ADR) associated with high mortality which requires immediate medical care. Carvedilol is a non selective adrenergic blocker used for the therapy of heart failure with hypertension and left ventricular dysfunction following Myocardial Infarction (MI) in clinically stable patients. Hereby, authors report of a 69-year-old male, with a history of MI and hypertension, who received Carvedilol at a dose of 75 mg daily and four days after the first dose of carvedilol, patient presented with erythematous maculopapular rash on face, trunk and limbs with fever up to 38°C. Skin biopsy from one of the fresh lesions showed apoptotic keratinoctyes, subepidermal cleft and monocytic infiltrate in the dermis and the findings were compatible with the diagnosis of TEN. Early recognition and cessation of the drug is of prime importance along with apt treatment and supportive care.

We aimed describe the chronic ocular sequelae of Kindler syndrome. All cases of Kindler syndrome with ocular involvement that presented to a tertiary eye care center were included. Three cases of Kindler syndrome with ocular changes were reviewed. Case 1 (10 years, female) had recurrent epithelial breakdown with severe dry eye and corneal opacity secondary to keratitis. Case 2 (28 years, male) had symblepharon , ocular surface keratinization , and severe dry eye. Case 3 (16 years , female ) had partial limbal stem cell deficiency with dry eye. All cases were treated with topical lubricants, short course of low-potency steroids and immuno-modulators. Attention must be paid to the eye in addition to the oro-an-genital mucosa to avoid longterm ocular sequelae. [ABSTRACT FROM AUTHOR]

Steven-Johnson syndrome (SJS) is a rare medical emergency that is characterized by widespread skin and mucosal lesions. Oral mucosal involvement is seen in 90% of SJS and it is often widespread and confluent. In the majority of the cases, the symptoms are associated with an adverse drug reaction, usually to non-steroidal anti-inflammatory drugs, sulfonamides, anti-epileptics, or antibiotics. Recently, SJS associated with herbal medicine has been reported. This paper aims to report 3 cases of SJS with marked oral manifestations, following the use of local herbal medicine, diagnosed in a children´s hospital in Nigeria within a year. Three children aged 1.5, 9 and 10 years presented in our emergency unit with features of SJS, history of the use of local herbal medicine to cure an ailment was elicited in all cases before admission into our hospital. All the cases presented with cutaneous exfoliating exanthema and bullous eruptions with oral mucosa appearing as painful crusted erythematous lesions. One of the 3 cases, a male patient, presented clinically with septicaemia complicated with anaemia as well as nasal involvement. Discontinuation of herbal medicine in all cases resulted in a relief of symptoms. A multidisciplinary approach of management was instituted with resultant total recovery of patients. This case report addresses the fact that severe hypersensitivity reactions can occur with the use of herbal medicine. Emphasis should be on active vigilance in the use of herbal medicine.

A 70 year old man presented with systemic signs of toxic epidermal necrolysis (TEN) following consumption of diclofenac tablets for a prodromal illness a week back. Ophthalmic evaluation showed no perception of light in both eyes along with lid edema, total corneal sloughing, and pus-filled anterior chamber. An amniotic membrane transplant was planned but within a few hours, both eyes developed panophthalmitis with restricted extraocular movements and mild proptosis and had to be eviscerated. This is perhaps the first case showing such devastating sequelae of TEN.

Latar Belakang: Steven Johnson Syndrome (SJS) dan Toxic Epidermal Necrolysis (TEN) merupakan suatu penyakit yang tergolong langka namun dapat mengancam nyawa. Ketidakjelasan etiologi, serta komplikasi yang mungkin timbul pada pasien anak dapat berdampak buruk terhadap prognosis penyakit. Tujuan: Menjelaskan gambaran klinis SJS, SJS – TEN overlap dan TEN pada pasien usia 0 – 18 tahun. Metode: Penelitian deskriptif retrospektif dengan menggunakan total sampling data rekam medis pasien anak usia 0 – 18 tahun di Instalasi Rawat Inap RSUD Dr. Soetomo Surabaya periode 2013 – 2016. Hasil: Terdapat 19 data rekam medis pasien yang memenuhi kriteria inklusi dengan rincian, 16 pasien dengan SJS, 1 pasien dengan SJS – TEN overlap, dan 2 pasien dengan TEN. Kesimpulan: Dugaan etiologi terbanyak adalah reaksi alergi yang diinduksi oleh obat jenis acetaminophen (24%), manifestasi klinis terbanyak adalah makula (100%), baik eritematosa dan hiperpigmentasi, penyakit penyerta terbanyak adalah malnutrisi (31%) dan konjungtivis (31%), komplikasi terbanyak adalah konjungtivitis (67%). Lama perawatan terbanyak adalah selama 1 hingga 7 hari (53%) dengan rata – rata lama perawatan selama 11,6 hari, konsultasi ke dokter lain terbanyak yaitu ke dokter spesialis mata (80%), dan tatalaksana terapi terbanyak adalah melakukan penghentian obat penginduksi timbulnya penyakit.

Steven Johnson syndrome is a severe cutaneous adverse reaction that develops especially against drugs and has an increasing incidence. The mortality rate in the elderly population is quite high compared to other age groups. Rapid diagnosis, early recognition, and discontinuation of the responsible drug reduce the mortality rate in patients admitted to the emergency department. The most important step in treatment is supportive treatment. We present a 70-year-old woman with a history of hypertension, coronary artery disease, known renal failure, and adrenal insufficiency. Steven Johnson syndrome developed. We emphasized that the use of allopurinol due to known renal failure and hyperuricemia increased the mortality rate by causing acute kidney damage, and after its rapid diagnosis and treatment, it positively affected mortality. The patient was admitted to the internal medicine service with a pre-diagnosis of Steven Johnson syndrome in the emergency department and was discharged with recommendations after 26 days of hospitalization. Because of the rapid spread and rapid deterioration of the general condition in severe cutaneous reactions such as Steven Johnson, mortality is important to make a rapid diagnosis, to determine the etiology, and to start treatment early. [ABSTRACT FROM AUTHOR]

Objective: To compare the cost of managing treatment-limiting cutaneous adverse drug reactions (CADRs) to first-line anti-tuberculosis drugs to an alternative strategy of immediate treatment initiation using second-line drugs in a South African setting.Methods: Clinical and cost data were retrospectively collected from patients presenting with a first-line anti-tuberculosis therapy-associated CADR. Costs (2016 US$) were estimated using an ingredient's approach from a healthcare provider perspective. The per-patient and total cost of drug rechallenge, the current management strategy for severe CADR, was calculated. Alternative strategies involving second-line treatment were derived from literature and expert clinical advice.Results: Drug rechallenge costs US $5831 (95% CI: 5134-6527) per patient. Hospitalisation accounted for 62% of this cost. Alternative CADR management strategies using regimens containing rifabutin, bedaquiline and/or delamanid cost 44%-55% less than drug rechallenge (US $2651-US $3276/patient). In univariate sensitivity analyses, drug rechallenge and alternative strategies were most sensitive to hospitalisation and tuberculosis drug costs, respectively.Conclusion: Cutaneous adverse drug reactions to anti-tuberculosis treatment represent a significant economic burden. An alternate strategy of outpatient-initiated second-line therapy is economically feasible but requires clinical validation to assess effectiveness. [ABSTRACT FROM AUTHOR]

A 70 year old man presented with systemic signs of toxic epidermal necrolysis (TEN) following consumption of diclofenac tablets for a prodromal illness a week back. Ophthalmic evaluation showed no perception of light in both eyes along with lid edema, total corneal sloughing, and pus-filled anterior chamber. An amniotic membrane transplant was planned but within a few hours, both eyes developed panophthalmitis with restricted extraocular movements and mild proptosis and had to be eviscerated. This is perhaps the first case showing such devastating sequelae of TEN. [ABSTRACT FROM AUTHOR]

Paracetamol is considered to be a relatively safe drug, even in the pediatric age group, at the recommended doses. Here we present a case of a six-year-old male presenting with symptoms and signs of Steven Johnson syndrome/toxic epidermal necrosis (SJS/TEN) following the ingestion of paracetamol. Steven Johnson syndrome/toxic epidermal necrosis is a potentially life-threatening dermatological emergency requiring intensive treatment. The patient was initially misdiagnosed as a case of chickenpox and was administered paracetamol. However, upon attending a tertiary care facility, he was diagnosed with TEN and treated with immunosuppressants. He recovered fully without any complications and was discharged within a week., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Jain et al.)

We present a 23-year-old white British male that presented to the Accident and Emergency Department, two weeks after the second dose of the BNT162b2 (BioNTech/Pfizer) vaccine. No similar use has been documented previously in literature. We report one potential complication of the Pfizer COVID-19 vaccine: A known case of Stevens-Johnson syndrome (SJS) that occurred after the second dose of the Pfizer COVID-19 vaccine alone without exposure to any other drug. Despite a significantly severe adverse drug reaction, the patient demonstrated completed recovery. The risk of developing severe cutaneous drug reaction from subsequent COVID-19 vaccinations in these patients is still unclear and remains a dilemma to date. [ABSTRACT FROM AUTHOR]

Objective: To identify the main factors causing Steven Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) and their clinical outcome in the patients in our local setup. Methodology: This case series was conducted at Lady Reading Hospital, Peshawar. A total of 54 consecutive patients who presented with Steven Johnson syndrome and toxic epidermal necrolysis over a two year period (2013-2015) were included in the study. Relevant information including bio-data, causative agents, duration of hospital stay and outcome in terms of improvement or mortality was collected. Analysis was done using SPSS version 20. Results: The mean age of patients presenting with SJS/TEN was 25.9±17.4 years with a minimum age of 1 year and a maximum age of 65 years. Majority of patients were between 18-45 years of age. The causes identified for triggering SJS/TEN were anticonvulsants (38.9%), followed in equal frequency by antibiotics and NSAIDS (27.8%) while 5.5% of cases were secondary to herpes virus infection. Patients had a variable course with 83.3% of patients improving with no sequel on follow-up and 16.7% succumbing to the disease. Conclusion: Anticonvulsants, antibiotics and NSAIDS were the most frequently responsible drugs for development of SJS & TEN. The Observed mortality rate was 16.7%. [ABSTRACT FROM AUTHOR]

Introduction: The diffuse epidermal exfoliation seen in Steven Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) is similar to skin loss in second degree burns, and many of these patients are referred for treatment at burn centers. Treatment can differ markedly from center to center, and mortality can range from 25% to 70%, including a considerable morbidity. However, our experience over a 15-year period from 2000 to 2015 with 40 patients found a mortality rate of only 10% (4/40). The purpose of this paper is to discuss our treatment algorithm as a model for other centers treating SJS/TENs patients.Methods: Records were reviewed for all patients admitted to the LAC+USC burn unit between 2000 and 2015 and 40 patients were identified with biopsy-proven SJS or TENS. These cases were reviewed for age, gender, initial and greatest TBSA, causative drug, pre-existing medical conditions, and morbidity and mortality. All data were entered into the SPSS statistical software package and all statistical analyses were performed using this program.Results: Our treatment algorithm focused on early referral to a specialty burn unit, immediate discontinuation of the offending drug, fluid resuscitation, nutritional supplementation, and meticulous wound care. Average time to transfer to a burn unit was 3.36 days. Silver-releasing antimicrobial dressings were applied to the affected skin surface and changed every 3 days. Mupirocin coated petroleum gauze was used for facial involvement. Steroids were tapered and discontinued if initiated at an outside facility (58% of patients), and starting after 2001, all patients received a course of IVIG. All patients received fluid resuscitation and the majority received supplemental tube feedings (69%). Average length of total stay was 17.1 days and length of ICU stay 15.9 days. While 44% were transferred to another facility for further rehabilitative care, 37% of patients discharge to home. In patients discharged home with complete resolution of skin lesions, time to healing was an average of 14 days.Discussion: With our 10% mortality rate in 40 patients, our study represents a relatively large study population while maintaining a relatively low mortality rate. The demographic data from our study largely aligns with the existing literature, and we therefore feel that our low mortality rate is due to our treatment algorithm, rather than to a less severe pathology in our patient population. This claim is supported by a standard mortality ratio of 1.68. This ratio proves a significantly improved mortality than would be expected based on disease severity on admission. [ABSTRACT FROM AUTHOR]

Psoriasis is a disease of the skin that is characterized by the presence of papular andscaly lesions. It is a clinical history of a woman of 38 years old who consulted forclinical manifestations of three days of evolution, consisting of sudden appearance oflesions such as erythematous, desquamative, confluents and widespread plaques thatreached to compromise more than 90% of the total body surface. These lesions wereof pruriginous and painful character. The patient had signs of systemic inflammatoryresponse given by leukocytosis and tachypnea. To check the case, medical record,analysis and laboratory exams of the patient, it was considered as Clinical- Impressions:(A) Pustular psoriasis of Von Zumbusch. (B) Steven Johnson Syndrome secondary to aningestion of Trimethoprim-sulfamethoxazole. Pustular psoriasis of Von Zumbusch is thevariant of generalized psoriasis with more risk of severe consequences. Rev.cienc.biomed. 2012;3(2):327-332RESUMEN:La psoriasis es una enfermedad de la piel que se caracteriza por la presencia de lesionespapuloescamosas. Se presenta la historia clínica de una mujer de 38 años de edad quienconsultó por cuadro clínico de tres días de evolución, consistente en aparición súbitade lesiones tipo placas, eritematosas, descamativas, confluentes y generalizadas quellegaron a comprometer más del 90% de la superficie corporal total. Estas lesiones erande carácter pruriginoso y doloroso. La paciente presentó signos de respuesta inflamatoriasistémica, dados por leucocitosis y taquipnea. Al revisar el caso, antecedentes, análisisy estudios de laboratorio de la paciente, se consideró como impresiones clínicas: (A)psoriasis pustulosa de Von Zumbusch, (B) Síndrome de Steven Johnson secundario aingesta de trimetropin-sulfametoxazol. La psoriasis pustulosa de Von Zumbusch es lavariante de la psoriasis generalizada con más riesgo de complicaciones severas. Rev.cienc.biomed. 2012;3(2):327-332

In everyday clinical practice, almost all physicians come across many instances of suspected adverse cutaneous drug reactions (ACDR) in different forms. Although such cutaneous reactions are common, comprehensive information regarding their incidence, severity and ultimate health effects are often not available as many cases go unreported. It is also a fact that in the present world, almost everyday a new drug enters market; therefore, a chance of a new drug reaction manifesting somewhere in some form in any corner of world is unknown or unreported. Although many a times, presentation is too trivial and benign, the early identification of the condition and identifying the culprit drug and omit it at earliest holds the keystone in management and prevention of a more severe drug rash. Therefore, not only the dermatologists, but all practicing physicians should be familiar with these conditions to diagnose them early and to be prepared to handle them adequately. However, we all know it is most challenging and practically difficult when patient is on multiple medicines because of myriad clinical symptoms, poorly understood multiple mechanisms of drug-host interaction, relative paucity of laboratory testing that is available for any definitive and confirmatory drug-specific testing. Therefore, in practice, the diagnosis of ACDR is purely based on clinical judgment. In this discussion, we will be primarily focusing on pathomechanism and approach to reach a diagnosis, which is the vital pillar to manage any case of ACDR.

Cutaneous adverse effects to antimicrobials are a major health problem. Though majority of them are mild and self-limiting, severe variants like Steven Johnson Syndrome (SJS), toxic epidermal necrolysis (TEN) are not uncommon. Ofloxacin, a fluoroquinolone widely used for the treatment of urinary tract infections, acute bacterial diarrheas, enteric fever, STDs and other soft tissue infections either as a single drug or in combination with other drugs. Earlier a case of mucocutaneous maculopapular rash with oral ofloxacin and was reported in an adult. In the present hospital set up there were few reports of such reactions to adults. Here we report three different variants of reactions associated with oral ofloxacin in chlidren. Early detection of cutaneous lesions and immediate withdrawal of the offending drug can prevent progression of such reactions to their severe variants as well as morbidity and mortality.

Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are severe, life threatening immunologically mediated, mucocutaneous, Adverse Drug Reactions (ADR) associated with high mortality which requires immediate medical care. Carvedilol is a non selective adrenergic blocker used for the therapy of heart failure with hypertension, and left ventricular dysfunction following Myocardial Infarction (MI) in clinically stable patients. Hereby, authors report of a 69-year-old male with a history of MI and hypertension, who received Carvedilol at a dose of 75 mg daily and four days after the first dose of carvedilol, patient presented with erythematous maculopapular rash on face, trunk and limbs with fever up to 38°C. Skin biopsy from one of the fresh lesions showed apoptotic keratinoctyes, subepidermal cleft and monocytic infiltrate in the dermis and the findings were compatible with the diagnosis of TEN. Early recognition and cessation of the drug is of prime importance along with apt treatment and supportive care. [ABSTRACT FROM AUTHOR]

Erythema Multiforme, (EM) an uncommon, acute inflammatory reactive mucocutaneous disorder and primary allergies confined to the oral mucosa. However the subsequent attacks can produce more severe forms of EM involving the skin. Manifestations of EM are varied and present a diagnostic dilemma because infections (particularly herpes simplex and mycoplasma pneumoniae) and drugs seem to predispose towards development of EM. We report two cases of EM in which drugs (Dioclofenac sodium & Amoxycilline) seems to be precipitating factor. In addition, the article reviews various aspects of EM as relevant to dental practice and highlight the associated potential etiologic agents, pathogenic mechanisms and therapies.

Erythema Multiforme, (EM) an uncommon, acute inflammatory reactive mucocutaneous disorder and primary allergies confined to the oral mucosa. However the subsequent attacks can produce more severe forms of EM involving the skin. Manifestations of EM are varied and present a diagnostic dilemma because infections (particularly herpes simplex and mycoplasma pneumoniae) and drugs seem to predispose towards development of EM. We report two cases of EM in which drugs (Dioclofenac sodium & Amoxycilline) seems to be precipitating factor. In addition, the article reviews various aspects of EM as relevant to dental practice and highlight the associated potential etiologic agents, pathogenic mechanisms and therapies. [ABSTRACT FROM AUTHOR]

Cutaneous adverse reactions associated with amlodipine have been rarely reported. Its most prevalent cutaneous side effect is flushing. Other recognized amlodipine associated skin eruptions, apart from urticaria and erythematous maculopapular rash which are most common allergic reactions, are rosacea and photodistributed telangiectasia which it is important not to misdiagnose as flushing, due to similarities in clinical presentations. Although these are usually mild to moderate skin side effects, they can cause serious discomfort and affect the patient's treatment compliance. Apart from these mild drug reactions, amlodipine use is associated with other more severe and nonpredictable skin disorders including drug induced vasculitis and Steven Johnson syndrome. These cutaneous adverse drug reactions are characterized with high morbidity and even possible lethal outcome. Although these reactions are in general rare and uncommon in the use of amlodipine, it is important to acknowledge them as a possible hypersensitivity syndrome induced by this drug. [ABSTRACT FROM AUTHOR]

Background: To study the ocular manifestation, complications and treatment of SJS & TEN in Rural Karnataka. Method: We studied the ocular manifestation and complications of SJS & TEN from March 2007 to October 2011. The details of the ocular examination and treatment were collected and examined to determine the pattern of presentation, complications, and treatment response. Results: A total of 33 patients, 10 males (30.31%) and 23 females (69.69%), were identified during the 4-year period. A majority of the patients (n=22; 66%) were between 20 and 40 years of age. All patients had bilateral involvement and most (n=33; 97.89%) had bilateral symmetrical presentation. The duration from the onset of symptoms to the time of presentation at the institute varied from 6 days to 1 year. 15 (45.45%) presented with in 10 days, 13(39.40%) presented within 2-6 months, five (15.15%) presented between 7-12 months. Intake of drugs was the most commonly identified possible etiology (n=20; 60.61%.8 patients (24.24%) had a prior history of viral infection with no history of drug ingestion. No definitive cause could be ascertained in 5 (15.15%) patients. The best corrected visual acuity (BCVA) was 6/12 or better in 14 (42.42%) patients. In the early presenters the main complications were lid edema (16.67%), conjunctival congestion (72.2%), and superficial punctate keratitis (83.3%). The late presenters had lid thickening (53.34%), entropion (26.67%), conjunctival xerosis (60%), symblepharon (20%), and there was scaring (33.33%), vascularization (33.33%) and thinning of cornea (6.67%). Conclusion: Ocular manifestations occur in a high proportion of patients with SJS/TEN. The most frequent causes were sulfonamide and nimesulide. A careful medication history should be obtained from these patients. Ophthalmic consultation, evaluation, and management are mandatory. Early diagnosis and intervention can prevent long-term squeal. [ABSTRACT FROM AUTHOR]

Nevirapine is a non-nucleoside reverse transcriptase inhibitor, widely used in combination with other antiretroviral agents for treatment of HIV infection. Steven Johnson syndrome (SJS) is the major toxicity of nevirapine. We describe here four cases of SJS in HIV seropositive patients following nevirapine therapy. In all four cases cutaneous hypersensitivity reaction was seen with extreme oral lesions, three patients presented clinically with elevated liver enzymes and hepatitis, and two patients had ocular involvement. [ABSTRACT FROM AUTHOR]

Abstract: Background: A retrospective study of the presentation, etiology, and prognosis of non-burn epidermal loss managed at the Lagos University Teaching Hospital Nigeria over a 12-year period. Materials and methods: Admission records of patients managed for non-burn skin loss were retrieved from the medical records. Demographic details of the patients, the initial diagnosis, final diagnosis, treatment and outcome of treatment was noted. Results: A total of 23 patients were identified, 17 (74%) had idiosyncratic drug reactions. Of this 17, 6 (26%) had Steven Johnson Syndrome, 6 (26%) had Steven Johnson Syndrome/toxic epidermal necrolysis while 5 (22%) presented with toxic epidermal necrolysis. Three of the five patients with toxic epidermal necrolysis died. The age range of patients with idiosyncratic adverse drug reactions was 2–28 years, mean, 10.18±1.44 years and male to female ratio of 1:1.83. The body surface area involved ranged from 8 to 78%; mean 26.65±6.08%. The agents suspected for the reactions were Co-trimoxazole (41.2%) and combination of Co-trimoxazole, and Fansidar® (17.6%). Other conditions seen were two (9%) Staphylococcal Scalded Skin Syndrome, three (13%) had Necrotizing Faciitis, one of whom was HIV positive and died. One (4%) patient presented with pemphigus vulgaris. The presentation and management of the patients was discussed. [Copyright &y& Elsevier]

Background and Objectives: We reviewed our case-load of patients with Toxic Epidermal Necrolysis (TEN) and analysed this oncologic disease in order to define the prevalence of this comorbidity and find eventual clinical and prognostic differences, specific of this subgroup of patients. Materials and Methods: We reviewed charts from January 1995 to December 2005. Only those patients with a TEN diagnosis proved with an histologic examination were included. Causative drugs, symptoms, management and outcome were recorded and analysed. Results: We found 32 patients with TEN and 9 of them (28%) had also cancer. The comparison among oncologic vs. the rest of patients showed no significant differences in age, delay of referral, % surface area epidermal detachment, blood chemistry, immunoglobulins therapy and bacterial isolation of species throughout the recovery (p > 0.05). Conclusions: Oncologic diseases were the most frequent comorbidities in our series. There were no differences in the length of stay, duration of disease or mortality between patients with and without cancer. However, due to the small number of patients, future larger prospective studies are necessary to confirm these findings. [ABSTRACT FROM AUTHOR]

Background and Objective: Toxic epidermal necrolysis and Steven Johnson syndrome are rare diseases that usually follow drug-exposures. The authors present one retrospective study with their management and focus their retrospective analysis on finding prognostic factors. Materials and Methods: We reviewed charts of admitted patients from January 1995 to December 2005. Only those with an histologic-proved diagnosis were included in the study. Causative drugs, symptoms, management and outcome were recorded and analysed. Results: We found 32 patients that met inclusion criteria. Mortality rate was 34.4% (11/32). Age, delay of referral, Total Burn Surface Area, white blood cells, creatinine, blood sodium, immunoglobulins therapy and more than two different types of blood bacterial species isolated were significantly correlated with death (p < 0.05). Conclusions: These data confirm prognostic factors already present in literature and find that the number of different bacterial species isolated from blood increase mortality. Further prospective studies are necessary to confirm these findings. [ABSTRACT FROM AUTHOR]

Background: The aim of this study was to describe the epidemiological, clinical and etiological aspects of severe cutaneous adverse drug reactions in children in dermatologyvenereology unit at National and Teaching Hospital of Cotonou., Methods: A retrospective and descriptive study was carried out for 10 years in dermatology-venereology unit at the National and Teaching Hospital of Cotonou to document the epidemiological, clinical and etiological aspects of severe cutaneous adverse drug reactions in children. It included all children aged from 0 to 18 years with clinical diagnosis of severe cutaneous adverse drug reactions. Drug imputability was based on the criteria of the French pharmacovigilance group., Results: Severe cutaneous adverse drug reactions accounted for 47.3% of paediatric cases (35/74 cases). The mean age was 9.3 years ± 5.2. The sex-ratio was 1.1. Self-medication was noted in 76.5% of children, on the initiative of parents in 66.7% of cases. There were 51.4% cases of Steven Johnson syndrome, 22.8% cases of Lyell syndrome, 8.5% cases of generalized and bullous fixed drug eruption, 2.9% cases of acute generalized exanthematous pustulosis and erythrodermic maculo-papular rash. Drug combinations was noted in 20% of cases. Penicillins (26.5%), paracetamol and sulfonamides (16.3%) were the drugs frequently incriminated., Conclusion: Steven Johnson syndrome and Lyell syndrome were the main severe cutaneous adverse drug reactions in children, mostly of school age. Penicillins, paracetamol and sulfonamides were the drugs frequently used and administered most often on self-medication., Competing Interests: The Authors declare that no competing interest exists., (Copyright © 2022 by West African Journal of Medicine.)

Mycoplasma pneumoniae  primarily causes atypical pneumonia in children and young adults. 7%-8% of patients with M. pneumoniae infections may experience extra-pulmonary manifestations, including M. pneumoniae -associated Stevens-Johnson Syndrome (SJS), also known as atypical SJS. In recent literature, there have been a few reports of isolated mucositis in children with M. pneumoniae infections. Due to significant overlap with several diseases, including autoimmune disease and infections, atypical mucositis associated with M. pneumoniae is often a diagnostic challenge. In addition, due to limited cases of M. pneumoniae -associated SJS, there is no established standardized treatment guideline that has been shown to reduce hospitalization duration and/or disease progression associated with  M. pneumoniae -associated SJS. We report a case of isolated mucositis in the absence of cutaneous involvement in a 10-year-old patient with an acute M. pneumoniae infection. Examination revealed erythematous ulcerations of his lips and pharynx with patchy exudates and bilateral submandibular lymphadenopathy. Laboratory investigation revealed a negative respiratory polymerase chain reaction (PCR) panel, which included M. pneumoniae . Further testing revealed a positive M. pneumoniae immunoglobulin M (IgM) titer on enzyme immunoassay. The diagnosis of atypical SJS was made secondary to M. pneumoniae . Treatment was initiated with systemic steroids and oral antibiotics. Limitations in diagnostic testing for M. pneumoniae in combination with non-specific clinical presentation make for challenges in confirming this pattern of SJS due to a primary M. pneumoniae infection. In this case, serological testing confirmed our suspected diagnosis, which guided treatment and helped reveal some of the difficulties in diagnosing and managing  M. pneumoniae -associated SJS., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Beheshti et al.)