Epidermal Necrolysis Overlap Research Articles

Stevens–Johnson syndrome-toxic epidermal necrolysis overlap in a patient taking quetiapine and famotidine: a case report

BackgroundStevens–Johnson syndrome-toxic epidermal necrolysis (SJS-TNE) overlap is a rare skin disorder characterized by erythema, blisters, extensive exfoliation, epidermal detachment, the involvement of multiple mucosae, and positive Nikolsky’s sign. SJS-TEN has a high mortality rate. Our case involves a rare occurrence of drug-induced Stevens–Johnson syndrome-toxic epidermal necrolysis overlap with a delayed onset in the setting of quetiapine and famotidine therapy.Case presentationAn 82-year-old Taiwanese female was admitted to our hospital for decreased urine output, generalized edema, and multiple skin blisters and bedsores. With further spread of the lesions, multiple ruptured bullae with shallow erosions on the face, trunk, and limbs and mucosal involvement affected 20% of the total body surface area. Nikolsky’s sign was positive. A diagnosis of Steven–Johnson syndrome was highly suspected. One month prior, she had started famotidine and quetiapine. Intravenous methylprednisolone treatment was initiated, which ameliorated the skin lesions after 3 days. However, new lesions developed after only 1 day of methylprednisolone tapering. The patient died 12 days after admission.ConclusionStevens–Johnson syndrome-toxic epidermal necrolysis is a rare skin disorder. Although it is mainly acute and has a high mortality rate, delayed onset can still occur. Quetiapine and famotidine are generally safe and effective for treating geriatric and gastrointestinal problems, but rare drug hypersensitivity reactions can lead to debilitating consequences. Therefore, increased clinical awareness and the initiation of supportive care are imperative. Optimal management guidelines are still lacking, and confirmation of developed guidelines through randomized controlled trials is needed. Collaboration for better management strategies is warranted.

Antibiotic-induced severe cutaneous adverse reactions: a single-center retrospective study over ten years.

Severe cutaneous adverse reactions (SCARs) are rare but life-threatening, with antibiotics being the main cause. This retrospective study from a single center was designed to analyze the culprit drugs, clinical features and treatment outcomes of antibiotic-induced SCARs. We analyzed cases of antibiotic-induced SCARs in a tertiary hospital in China between January 2013 and January 2024, including Steven-Johnson syndrome (SJS) or Stevens-Johnson syndrome-toxic epidermal necrolysis (SJS-TEN) overlap, toxic epidermal necrolysis (TEN), drug reaction with eosinophilia and systemic symptoms (DRESS) and acute generalized exanthematous pustulosis (AGEP). Descriptive analysis of the demographic characteristics, clinical manifestations, treatment and prognosis were carried out. Among 354 cases of SCARs, 63 validated antibiotic-related cases were included. Cephalosporins (31.7%), penicillins (25.4%), and quinolones (19.0%) were the most common triggers for SCARs. Overall, liver (50.8%), lungs (31.7%), and kidneys (23.8%) were the most frequently affected organ in SCARs cases. Eight patients (28.6%) in the SJS/SJS-TEN overlap group and 8 patients (80.0%) in the TEN group received combination therapy of corticosteroids and IVIG. Patients with SCARs caused by penicillins or cephalosporins could receive alternative treatments such as lincomamides, quinolones, and tetracyclines. The mortality rate in the TEN group was the highest at 20.0%, followed by the SJS/SJS-TEN overlap group (7.1%), and no deaths were observed in the DRESS and AGEP groups. The identification of the culprit antibiotics and the application of alternative antibiotic therapies are crucial for the management of antibiotic-induced SCARs. If complicated underlying conditions and complications like advanced age, cancer and pneumonia coexist with SCARs, patients might be more at risk for mortality.

A case report of Steven Johnson’s Syndrome (SJS) /Toxic Epidermal Necrolysis(TEN) overlap caused by Nimesulide

Introduction: Stevens–Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are rare, acute and life-threatening mucocutaneous diseases that are nearly always drug-related. Incidence of SJS and TEN is 0.05 to 2 persons per million populations per year. In India, Nimesulide is easily available as an over the counter drug (OTC), different cutaneous reactions associated with, Nimesulide including angioedema, maculo-papular rash, severe urticaria, pityriasis rosea and worsening of preexisting psoriasis. Patient concern (Case summary): A 17 years old female patient complained about fever and she was prescribed with Tab.Nimesulide twice daily by private practitioner. On the 3rd day of intake of oral Nimesulide, patient developed skin lesions over face, trunk, back and all four limbs. This reaction was diagnosed as Stevens–Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) overlap by Nimesulide based on clinical picture and drug history. Interventions: Tab. Nimesulide was stopped and patient was admitted in dermatology ward. Patient was treated with Inj.Hydrocortisone sodium succinate 100mg iv twice daily, Inj.Cefotaxime 1gm iv thrice a daily, Inj. Metronidazole 500mg once a day and Liquid Cyclosporine 50mg twice a day for 10days. Other treatments included Inj. Albumin 100ml i.v slowly for 3 days given. Patient recovered and was discharged after 10 days. Conclusion: We are presenting a case of SJS-TEN overlap caused by Nimesulide, which is commonly used as an over the counter drug (OTC). Our aims to minimize this type of serious ADR by making alert to physicians and other healthcare professionals, we can avoid hospital admissions because of ADR, reduce economic burden of the patients and health related quality of life of the patient can be improved.

Amikacin induced Stevens Johnson syndrome and toxic epidermal necrolysis overlap: a rare case report

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are the most severe adverse drug reactions. They are characterized by necrosis and epidermal release in vesicobulosa skin, mucous orifice, and eyes, with more severe general symptoms. SJS/TEN-overlap syndrome is the term used to characterize situations where 10–30% of the body skin area is detached. TEN or SJS is one of the deadliest dermatological catastrophes. Despite being a rare condition, it frequently has a high death rate. Different types of purpuric macules or rounded patches with mucosal lesions are characteristic features of these type 3 hypersensitivity reactions. We are presenting a case of 50-year-old female patient brought to hospital on account of multiple black to red coloured raised lesions over body in the last 8 days. Patient was asymptomatic in the last 8 days after that patient noted red coloured lesions first over face followed by lower back, upper limb, lower limb followed by itching and Jiburning sensation all over the body. The patient had symptoms such as redness of eyes in the last 3-4 days, burning sensation and ulcers in mouth in the last 3-4 days. These symptoms developed day after she received amikacin. She was diagnosed with SJS-TEN overlap. This case highlights the precipitant of antibiotics for SJS. She was recovered completely after stopping the causative drug and treatment with Immunoglobulin with other symptomatic measures. The causative drug was found to be Amikacin and medicine taken for comorbid conditions like epilepsy, hypothyroidism and hypertension. Rarely, amikacin has been linked to a range of drug hypersensitivity reactions, including toxic epidermal necrolysis, Stevens-Johnson syndrome, and overlap between the two conditions.

Severe cutaneous adverse reactions in a tertiary care center in Jamaica

BackgroundSevere cutaneous adverse reactions (SCARs) are associated with morbidity and mortality. ObjectiveThe aim was to determine the different types of SCARs, their morphology, common offending drugs, interventions, and outcomes. MethodsA retrospective cohort study was conducted of all patients admitted to the dermatology service at the University Hospital of the West Indies with Stevens-Johnson syndrome (SJS), Stevens-Johnson syndrome/toxic epidermal necrolysis overlap (SJS/TEN), toxic epidermal necrolysis (TEN), drug reaction with eosinophilia and systemic symptoms (DRESS) and acute generalized exanthematous pustulosis (AGEP) between January 1, 2012 to June 1, 2022. ResultsFifty-one cases (51) met the inclusion criteria for SCAR. SJS, SJS/TEN overlap and TEN together accounted for 71.2% of cases. SCARs were most frequent in the 4th, 5th and 6th decades of life and there was a female preponderance.Antibiotics (31%) and anticonvulsants (29%) were the most common causative agents for SCARs. Most patients had at least one complication. The liver was the most common extracutaneous organ affected.Mortality was 7.8%. The main cause of death was sepsis. LimitationsResults were not generalizable. There was missing data and loss to follow-up. ConclusionJudicious use of antimicrobials and corticosteroids may be beneficial in treatment of severe cutaneous drug reactions.

Pathogenesis and A Practical Guide to the Management of Steven-Johnson Syndrome & Toxic Epidermal Necrolysis

Steven Johnson Syndrome (SJS) and Toxic Epidermal necrolysis are rare dermatological emergencies that are associated with a high degree of morbidity and mortality [1]. They are considered to be severe blistering conditions that portray an image of diffuse epidermal necrolysis in association with sloughing of the skin. SJS and TEN are effectively the same disease with the main difference being the percentage of body surface area involved. It is widely accepted that SJS belongs to the below 10% body surface area category and TEN to the more than 30% category. A Steven Johnson syndrome and Toxic Epidermal Necrolysis overlap exists when the body surface area affected is between 10 percent and 30 percent [2]. The causes of both conditions within this spectrum are most likely drug related, however certain infections can also trigger SJS/TEN but this is less likely. In a substantial number of cases the cause is idiopathic [3]. The main grading system is the SCORTEN criteria, which can also provide more information regarding prognosis and mortality [4]. Management of this condition is highly controversial with no clear guidelines but there is a wide agreement that patients should be managed in a burns unit with intravenous fluids and protection from secondary infections. Stopping all medications and investigating for the cause is important unless there is clear benefit from any single medication.

Steven-Johnson Syndrome/ Toxic Epidermal Necrolysis Overlap Complications

ABSTRACT Steven–Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) overlap is a life-threatening disorder which can lead to mortality because of systemic complications. Here, we present a case of a 22-year-old female referred to the hospital with generalized epidermal detachment and necrolysis covering approximately 25% body surface area, and unstable vital signs were found. Laboratory examination results showed acute renal failure (ARF), anemia, and hypoalbuminemia. Comprehensive treatment of skin and systemic conditions must be carried out to avoid mortality and improve the outcome. This case highlights a case of SJS overlap TEN with shock, ARF, anemia, and hypoalbuminemia safely treated by a conservative treatment strategy.

Stevens-Johnson syndrome/toxic epidermal necrolysis overlap triggered by ethosuximide: a rare pediatric report

Stevens-Johnson syndrome/toxic epidermal necrolysis overlap triggered by ethosuximide: a rare pediatric report

Combination of cyclosporine A and methylprednisolone to treat pediatric Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome.

The treatment of epidermal necrolysis in pediatric patients remains a major challenge. Cyclosporine A has emerged as a promising therapy for epidermal necrolysis in adults; however, its efficacy in children is unclear. We present the case of a boy with Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome who was initially resistant to methylprednisolone monotherapy but improved after receiving the combination of cyclosporine A and methylprednisolone. Published reports on the use of cyclosporine A for pediatric epidermal necrolysis are also briefly reviewed.

Drug Reaction with Eosinophilia and Systemic Symptoms Syndrome with Toxic Epidermal Necrolysis: Overlap in an HIV Seropositive Male with Diffuse Large “B” Cell Lymphoma – A Rare Case Report

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a severe drug-induced hypersensitivity reaction with varied presentations. We report a rare case of DRESS syndrome with toxic epidermal necrolysis (TEN) overlap associated with non-Hodgkin lymphoma (NHL). A 52-year-old male with retroviral disease presented with fever and diffuse skin peeling with mucosal erosions. The patient was on antitubercular therapy (ATT) for 1 month for suspected tubercular cervical lymphadenitis. He had facial edema, extensive skin tenderness, and denudation with a positive Nikolsky’s sign, icterus, hepatosplenomegaly and multiple enlarged immobile nontender cervical, and axillary and inguinal lymph nodes. Investigations showed leukocytosis with hepatic and renal dysfunction. Skin histopathology was consistent with TEN. Based on Registry of Severe Cutaneous Adverse Reaction criteria, a diagnosis of ATT-induced DRESS with TEN overlap was made. Cervical lymph node biopsy and immunohistochemistry confirmed diffuse large B-cell NHL. ATT was withheld and the patient was treated with systemic steroids and assessed for initiation of chemotherapy. The profound immunological dysregulation in individuals with retroviral disease leads to varied clinical presentations, increasing the chances of diagnostic errors, and culminating in challenging scenarios. Therefore, seemingly innocuous symptoms and signs warrant thorough evaluation. Sound clinical acumen and prompt investigations for unusual causes such as malignancies can improve outcomes by aiding accurate diagnosis as well as avert untoward complications such as DRESS syndrome/TE.

Stevens–Johnson/Toxic epidermal necrolysis overlap syndrome in paediatric age

Stevens–Johnson/Toxic epidermal necrolysis overlap syndrome in paediatric age

Toxic Epidermal Necrolysis and Stevens-Johnson Syndrome or Toxic Epidermal Necrolysis Overlap in Pediatric Patients With a Focus on Newer Antiepileptic Drugs: A 25-year Retrospective Study at a Single Tertiary Care Center

Toxic Epidermal Necrolysis and Stevens-Johnson Syndrome or Toxic Epidermal Necrolysis Overlap in Pediatric Patients With a Focus on Newer Antiepileptic Drugs: A 25-year Retrospective Study at a Single Tertiary Care Center

Acetaminophen induced Stevens Johnson syndrome - Toxic Epidermal Necrolysis Overlap Syndrome: A rare Adverse Reaction

Stevens Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are hyper sensitive life - threatening rare dermatological reactions. In SJS - TEN overlap syndrome, the extent and severity of epidermal detachment is 10- 30%. Drugs like sulfonamides, nonsteroidal anti- inflammatory agents, antibiotics and anticonvulsants are the commonest precipitants. Here, we present a rare case of SJS - TEN overlap syndrome in a previously normal 11 years old girl after acetaminophen intake for fever. This case report is intended to make paediatricians aware that the adverse reaction like SJS - TEN overlap syndrome can develop with a drug like acetaminophen which is supposed to be the safest antipyretic in paediatrics.

Stevens-Johnson syndrome/toxic epidermal necrolysis overlap following sulfadoxine-pyrimethamine overdose: a case report

Stevens-Johnson syndrome/toxic epidermal necrolysis overlap is a rare but severe delayed-type hypersensitivity reaction that presents with widespread blistering, ulceration and necrosis of the skin and mucosa in the genetically predisposed and leads to significant morbidity and mortality. Sulfadoxine-pyrimethamine has been reported to cause a spectrum of drug hypersensitivity reaction (Stevens-Johnson syndrome, toxic epidermal necrolysis or Stevens-Johnson syndrome/toxic epidermal necrolysis overlap), but in rare occasions, hence this report. We report a 5-year-old boy who presented with fever and extensive mucocutaneous blistering and ulcerations 10 days following the ingestion of an overdose of sulfadoxine-pyrimethamine administered by a community health extension worker. Skin involvement was about 19%. The Naranjo Adverse Drug Reaction Probability Scale score of 6 was assigned (implying probable association between the ingested drug and the hypersensitivity reaction). The severity-of-illness score of toxic epidermal necrolysis of 1 was also assigned to this patient (implying low severity). He was managed conservatively with fluid, antibiotics, nutritional support and wound care measures. He recovered completely and satisfactorily. The patient's sister and father had reacted similarly but less severely when exposed to sulfadoxine-pyrimethamine. Health workers should pay close attention to every patient's family drug allergy history before administering any medications. Also, basic pharmacogenetic testing should be introduced into health facilities, especially for drugs known to cause serious adverse drug reactions. Furthermore, health workers should keep abreast with updates on treatment guidelines and also dutifully report all cases of adverse drug reactions to relevant bodies.

Concomitant Nicolau’s and Stevens-Johnson syndrome—Toxic epidermal necrolysis overlap after diphtheria, pertussis, tetanus vaccination: An unusual presentation in a young child

Diphtheria, pertussis, tetanus (DPT) is a live attenuated vaccine included in the national immunization schedule. Common adverse effects of this vaccine include localized pain, erythema, and high-grade fever. Rarely, it can cause severe localized or generalized reactions such as Nicolau’s syndrome (NS), erythema multiforme (EM), Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN). A 6-year-old boy developed severe pain over left gluteal region, immediately after DPT vaccine, followed by local ulceration. Two days later, he also developed mucocutaneous lesions. Clinical features were consistent with NS and SJS-TEN overlap. Concomitant NS with SJS-TEN overlap after DPT vaccine is rare and has not been reported earlier.

Oxcarbazepine-induced stevens johnson syndrome and toxic epidermal necrolysis overlap

Oxcarbazepine-induced stevens johnson syndrome and toxic epidermal necrolysis overlap

Dietary Collagen Supplements Might Not Be Completely Innocent: A Case of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Overlap Induced by a Collagen Supplement.

Dietary Collagen Supplements Might Not Be Completely Innocent: A Case of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Overlap Induced by a Collagen Supplement.

Cutaneous adverse reactions associated with antiseizure medication: clinical characteristics and implications in epilepsy treatment.

To describe the clinical characteristics of cutaneous adverse reactions and cross-sensitivity induced by antiseizure medications and compare the pattern of use of antiseizure medications in patients with epilepsy according to skin rash history. We analysed patients with a history of skin rash presenting for up to 12 weeks after initiating antiseizure medication. The history of skin rash was verified by medical charts, interviews, and identification of skin lesions by patients based on illustrative images. The minimum follow-up period was eight months. The control group comprised epilepsy patients with regular antiseizure medication use for at least 12 weeks without skin rash. We included 109 cases and 99 controls. The median (interquartile range) period from the index rash was six years (2-11). Carbamazepine was the trigger medication in 48% of cases and induced skin rashes in all patients with cross-sensitivity and carbamazepine exposure. Stevens-Johnson syndrome, toxic epidermal necrolysis, or drug reactions with eosinophilia and systemic symptoms affected 36% of cases. Carbamazepine- or oxcarbazepine-induced maculopapular exanthema occurred earlier (median: one week) than that induced by other antiseizure medications (median: three weeks) (p=0.006). Cross-sensitivity was more common in patients with at least one episode of Stevens-Johnson syndrome (29%) and Stevens-Johnson/toxic epidermal necrolysis overlap (50%) than in patients with maculopapular exanthema (8%) (p=0.01). Although most cases were mild, the pattern of antiseizure medication use differed from that of controls, with a lower proportion of antiseizure medication typically associated with severe cutaneous adverse reactions (carbamazepine, phenytoin, phenobarbital, primidone, oxcarbazepine, and lamotrigine) (p<0.001). Most cases exposed to high-risk medication, however, did not develop cross-sensitivity. Cutaneous adverse reaction history may influence antiseizure medication use. Cross-sensitivity is more common in severe cases and most patients are affected by mild, self-limited skin rashes. Further research should consider the relevance of mild skin rashes in lifelong epilepsy treatment.

Toxic epidermal necrolysis and Stevens-Johnson syndrome/toxic epidermal necrolysis overlap in pediatric patients with a focus on newer antiepileptic drugs: A 25-year retrospective study at a single tertiary care center.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis Syndrome (TEN) are rare immune-mediated diseases. Extensive research on adult triggers of SJS and TEN is available; however, research in children is more limited. We sought to investigate and report the experience with pediatric SJS and TEN in our center, identifying associated medications. A retrospective review from 1990 to 2015 at the Shriner's Burn Hospital in Galveston, Texas was performed to identify patients diagnosed with SJS, SJS/TEN overlap, and TEN. Data pertaining to demographic characteristics, medical history, physical exam, treatment, and outcomes were collected. We identified SJS/TEN overlap or TEN in 51 patients. Antiepileptic drugs were the most common group of causative agents, closely followed by antibiotics. The most common causative agents were trimethoprim-sulfamethoxazole, phenytoin, and lamotrigine used concomitantly with valproic acid. Newer generation agents, with the definition of agents approved after 1990, were the cause in 13/51 (25.5%) cases. Newer generation agents included lamotrigine, clobazam, and zonisamide. Seven patients died, resulting in a 13.7% mortality rate. Renal failure, liver failure, sepsis, and gastrointestinal involvement each had a statistically significant association with mortality. SCORTEN was statistically significantly greater in patients who died compared to children who lived (3 vs 2). This is a retrospective study. Three drugs introduced into the market since 1990 have emerged as causes of SJS/TEN overlap and TEN: lamotrigine, clobazam, and zonisamide. These medications are being used more widely to treat seizures, as well as mood disorders. It is also important for clinicians to be aware of the extremely commonly used medications such as amoxicillin, tetracyclines, NSAIDs, and acetaminophen that can rarely cause SJS and TEN.

A case report on Deflazacort Induced Stevens Johnson Syndrome/Toxic Epidermal Necrolysis Overlap

A case report on Deflazacort Induced Stevens Johnson Syndrome/Toxic Epidermal Necrolysis Overlap