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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]

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]

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]

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]

Steven Johnson Syndrome is an acute life threating, fatal drug reaction presenting as severe mucosal erosions with widespread erythematous, cutaneous macules or atypical targets. Majority of the cases are drug induced affecting oral and perioral region. A case of SJS/TEN overlap following treatment with phenytoin is being presented here. A 30 year old male was put on phenytoin for epilepsy; he developed rashes, vesicle, bullae all over body associated with pain, itching, fever and purulent discharge from both eyes and ears after 10 days of treatment. The patient was managed by withdrawing of phenytoin immediately and treated with systemic corticosteroids, antimicrobial, antifungal and antihistamines. This case has been presented here to highlight the necessity of judicious use of widely prescribed antiepileptic phenytoin to prevent life threatening adverse drug reaction. [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]

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]

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. [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]

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]

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. [ABSTRACT FROM AUTHOR]

Steven Johnson syndrome (SJS) is a rare drug induced mucocutaneous reaction. Here, we present an elaborate report of a 28-year-old female patient who developed Phenytoin induced SJS, which was exacerbated by cefepime. [ABSTRACT FROM AUTHOR]

Trimethoprim-sulfamethoxazole (TMP/SMX) is a widely prescribed antimicrobial for the management of several uncomplicated infections. It is commonly used for the treatment and prophylaxis of Pneumocystis jirovecii pneumonia (PCP) in the HIV-infected population. The adverse reaction to TMP/SMX is more frequent and severe in HIV-infected patients as compared to the general population. Here, we report a case of Stevens-Johnson syndrome (SJS) secondary to TMP/SMX. The patient had a generalized cutaneous reaction with involvement of the eyes, oral cavity, and genitals. He had elevated hepatic alanine aminotransferase and aspartate aminotransferase enzyme. TMP/SMX therapy was stopped and supportive treatment was started. His condition improved after eight days of stopping TMP/SMX therapy. [ABSTRACT FROM AUTHOR]

Purpose: To report three cases of bilateral microbial keratitis in eyes with Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) induced by highly active antiretroviral therapy (HAART) in patients of acquired immune deficiency syndrome (AIDS). Methods: A case series. Results: A detailed clinical examination and systemic review of all the three patients on HAART was performed. While one manifested with the more severe variant of TEN, two of these patients presented with SJS with ocular involvement. Despite withdrawal of nevirapine, the ocular surface disorder persisted. The entailing chronic epitheliopathy along with the compromised immune status led to the development of secondary microbial keratitis in all these cases. Conclusions: The immune reconstitution occurring as a response to the antiretroviral therapy may potentially increase immunologically mediated diseases like SJS and TEN, which in turn may predispose the eye to develop corneal ulcer. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]