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Abstract Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe mucocutaneous disorders characterized by extensive tissue necrosis; they are often accompanied by severe ocular complications (SOC). The regulatory role of microRNAs (miRNAs) in modulating immune responses in SJS/TEN is not fully understood, particularly in relation to chronic SOC. We explored the expression profiles of specific miRNAs and their potential impact on the regulation of key innate immune genes in patients with SJS/TEN with SOC. We analyzed plasma samples from 100 patients with chronic stage SJS/TEN with SOC and 92 healthy controls to examine the expression levels of eight specific miRNAs (let-7a-5p, let-7d-3p, let-7e-5p, miR-146a-5p, miR-130a-3p, miR-151a-3p, miR-151a-5p, miR-27b-3p) using quantitative RT-PCR (RT-qPCR). In addition, we subjected mononuclear cells from 12 SJS/TEN patients and 9 controls to RT-qPCR to assess the expression of the innate immune-related genes IFI44L, TNFSF10, AIM2, RSAD2, CXCL10, TRIM22, IFI27, and IFIT2. Significant upregulation of 4 miRNAs (let-7a-5p, let-7e-5p, miR-146a-5p, and miR-27b-3p) was observed in the plasma of SJS/TEN patients; this correlated with the increased expression of TLR3, RIG-I, and MDA5. Furthermore, MDA5, IFI44L, RSAD2, CXCL10, and IFIT2 were also significantly up-regulated in the mononuclear cells from these patients, indicating a systemic modulation of immune response genes. Our findings demonstrate that specific miRNAs are up-regulated in SJS/TEN with SOC and associated with the upregulation of critical immune response genes, suggesting their involvement in the pathogenesis and persistence of SOC. These miRNAs and their target genes may serve as potential biomarkers or therapeutic targets in managing SJS/TEN with SOC.

Abstract Background To present a case of conjunctival growth on the amniotic membrane and subsequent pathology revealing conjunctival squamous metaplasia in a patient with Stevens-Johnson syndrome. Case presentation A 21-year-old female presented with painful, blurred vision in both eyes for two weeks. She was diagnosed with Stevens-Johnson syndrome 5 weeks before. Due to bilateral corneal epithelial defects, ProKera®, an amniotic membrane corneal bandage with a polycarbonate ring, was placed in both eyes. However, three weeks later, a slit-lamp examination revealed vascularized tissue growth from the palpebral conjunctiva to the amniotic membrane, along with symblepharon formation in the left eye. The patient underwent conjunctival biopsy, amniotic membrane removal, and symblepharon release. Pathology report showed the growth of squamous epithelium on the acellular amniotic membrane. Immunohistochemistry further supported the diagnosis, revealing squamous markers through p40 staining and highlighting the presence of the amniotic membrane using trichrome stain. Three months later, the patient’s visual acuity had improved to 20/25 and no symblepharon was noted. Conclusions This is the first case of conjunctival squamous metaplasia on amniotic membrane associated with Stevens-Johnson syndrome. Our case indicates that, despite the anti-inflammatory properties of amniotic membrane, conjunctival squamous metaplasia may arise after amniotic membrane grafting due to intense inflammation in Stevens-Johnson syndrome. Clinicians should conduct regular monitoring before amniotic membrane dissolution to preclude the development of conjunctival squamous metaplasia on the membrane and potential invasion into the cornea.

Abstract Background Stevens–Johnson syndrome (SJS) is a life-threatening condition characterized by high fever and severe mucocutaneous lesions, often triggered by drugs or infection. During the coronavirus disease 2019 pandemic, there was a marked increase in Stevens–Johnson syndrome cases, but relatively few cases were reported in children. The present article reports a pediatric case of Stevens–Johnson syndrome due to coronavirus disease 2019 infection and provides a review of the most relevant literature. Case presentation A previously healthy 15-year-old Han Chinese boy from China presented to the hospital with oral ulcers, conjunctival hyperemia, and widespread maculopapular rash. He had a history of fever 9 days prior and tested positive for coronavirus disease 2019 infection. Upon admission, his rash and mucosal lesions worsened, with the development of blisters on the fingertips of both hands, ocular pain, photophobia, and erosive lesions on the genital mucosa with exudation. He was diagnosed with Stevens–Johnson syndrome and received treatment with methylprednisolone, intravenous immunoglobulin, and dermatological and mucosal care. The patient’s condition was managed, and the dosage of high-dose intravenous methylprednisolone was tapered down, followed by a transition to oral prednisolone. He was discharged without sequelae. Conclusion We should be aware that coronavirus disease 2019 infection is associated with the development of Stevens–Johnson syndrome in children and may lead to a wide spectrum of dermatologic presentations. Although Stevens–Johnson syndrome is a relatively rare condition, given its potentially serious consequences, it is crucial to identify it as early as possible and to take appropriate preventive and therapeutic measures to reduce complications and improve the quality of life for patients.

Background: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rarely described in the pregnant population, and knowledge of their impact on the mother/fetus is limited. Objective: To describe SJS/TEN in pregnant women and to investigate the risk factors for developing SJS/TEN in pregnancy. Methods: We utilized hospitalization data from the 2009–2020 National Inpatient Sample. Pregnancy hospitalizations and SJS/TEN involvement were identified by ICD-9/10 codes and analyzed by chi-square and logistic regression. Results: We identified 650 pregnancies complicated by SJS/TEN requiring hospitalization. The median age was 28 years, and most were non-Hispanic White (55.2%). There were ≤10 cases associated with mortality. Most SJS/TEN cases (73.9%) occurred during the third trimester. HIV infection (OR = 9.49; P = .030), herpes simplex virus infection (OR = 2.49; P = .021), genitourinary tract infections (OR = 3.80; P

Abstract Background Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but life-threatening skin lesion triggered by hypersensitive drug reaction. They are characterized by extensive epidermal necrosis and skin exfoliation. Fulminant type 1 diabetes mellitus (FT1DM) is featured by a rapid-onset of hyperglycemia with ketoacidosis due to severely destroyed β-cell function. Fulminant type 1 diabetes mellitus as a sequela of SJS/TEN has rarely been reported. Case presentation We present a 73-year-old female patient who developed SJS/TEN skin allergic reaction after taking carbamazepine and phenytoin for 35 days. Then, hyperglycemia and diabetic ketoacidosis occurred 20 days after discontinuation of antiepileptic drugs. A very low serum C-peptide level (8.79 pmol/l) and a near-normal glycosylated hemoglobin level met the diagnostic criteria for fulminant T1DM. Intravenous immunoglobulin (IVIG) and insulin were promptly administered, and the patient recovered finally. Conclusions This rare case indicates that monitoring blood glucose is necessary in SJS/TEN drug reaction, and comprehensive therapy with rehydration, insulin, antibiotics, and IVIG may improve the prognosis.

Objective To summarize and analyze the skin care methods for and outcomes of patients with Stevens-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN). Methods Inpatients with SJS or TEN at Dermatology Hospital of Southern Medical University from January 2016 to February 2023 were retrospectively analyzed. The changes in the total body surface area (BSA), control time, healing time, hospitalization days and complications of skin lesions during hospitalization were analyzed. Results Forty-one patients were included. Fourteen patients were treated with dry powder exfoliation, while 16 patients were treated with wet-dress healing care. The rest 11 cases were treated with the combination of dry powder exfoliation, moist burn ointment and vaseline gauze. On the 5th day, treatment with either dry powder exfoliation or wet-dress healing care significantly decreased the BSA (t=5.25,6.28, P

ABSTRACT Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe mucocutaneous reactions, often triggered by medications, characterized by blistering and epithelial sloughing. We report the case of a 66‐year‐old male who presented with a 2‐day history of fluid‐filled lesions on his body. On examination, erosions were observed on the posterior and anterior trunk, as well as on both upper and lower limbs. Multiple vesicles and bullae were scattered bilaterally, involving 60%–70% of the body surface area. Co‐trimoxazole‐induced SJS was diagnosed. The patient was admitted to the ICU and treated with dexamethasone, hydrocortisone, imipenem, and azithromycin. Corticosteroids, combined with broad‐spectrum antibiotics, were effective in managing the condition. Early intervention and a multidisciplinary approach helped prevent complications and secondary infections.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but serious cutaneous reactions characterized by epidermal and mucocutaneous detachment, most often drug-induced. SJS and TEN are considered the opposite extremes of the same spectrum of disease, where the percentage of skin involvement is 30% in TEN; the in-between range is called a SJS/TEN overlap. We present the case of a 64-year-old patient who was treated with lamotrigine, an anti-epileptic drug, and developed SJS/TEN. After being hospitalized and recovering for three days due to the worsening of the clinical presentation, he was transferred to a burn center. Making an early diagnosis and identifying the indicated drug is extremely important to set the appropriate treatment and reduce mortality. Advanced supportive care is required.

We reported a 10-year-old child who suffered from severe toxic epidermal necrolysis triggered by trimethoprim–sulfamethoxazole and managed successfully with multi-targeted therapy. He was jointly managed by a paediatric intensivist, a dermatologist, an otolaryngologist, a urologist, a wound nurse, a pain management specialist, a dietitian, and a clinical psychologist. Systemic intravenous immunoglobulin and pulsed-dose methylprednisolone were initiated after admission. Oral cyclosporin A was added in the early stage of the disease in view of severe ocular involvement with progressive inflammation of bilateral upper and lower eyelids, the presence of pseudomembrane, diffuse conjunctival injection, and progression of central epithelial defects in bilateral eyes. He underwent amniotic membrane transplantation. Subcutaneous injection of etanercept was added on the treatment to allow rapid tapering of steroids. Finally, the disease progression was halted with re-epithelisation on day 13. He experienced no side effects from the multi-targeted therapy and recovered well without clinical sequelae.

Abstract Background Stevens–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 presentation An 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. Conclusion Stevens–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.

Recently, there has been discussion to reclassify pediatric Stevens–Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) as drug-induced epidermal necrolysis (DEN), separating it from infectious etiologies and redefining pediatric mucocutaneous eruptions as either reactive infectious mucocutaneous eruption (RIME) or DEN. In this report, we describe a previously healthy 4-year-old girl with rapidly progressive mucocutaneous blistering involving four mucosal membranes and 37.5% of total body surface area (BSA) following a prodromal rhinovirus and enterovirus infection. The symptoms occurred in the absence of an inciting medication and improved with only supportive care. This case illustrates a rare occurrence of RIME with TEN-like BSA involvement, prompting a review of the literature exploring the relationship between BSA involvement in RIME and its influence on patient outcomes. Findings support the proposed reclassification of SJS/TEN as DEN and postinfectious mucocutaneous eruptions as RIME.

Mycoplasma pneumoniae is an atypical bacterium causing respiratory tract infections mainly in the pediatric population. As a superantigen, it dysregulates the immune system and promotes immunological reactions. Dermatological symptoms occur in approximately one-fourth of the patients infected with this bacterium. This review describes skin diseases occurring during Mycoplasma pneumoniae infection. Differences in the course of these diseases compared to their presentation associated with other etiological factors, are also discussed. Among the cutaneous manifestations of Mycoplasma pneumoniae infection, unspecific rashes and urticaria are the most common. This bacterium is also a frequent cause of erythema multiforme, Stevens-Johnson syndrome, Mycoplasma-induced rash and mucositis, and erythema nodosum. Less frequently toxic epidermal necrolysis, leukocytoclastic vasculitis, IgA vasculitis, subcorneal pustular dermatosis, Gianotti-Crosti syndrome, and Sweet syndrome are described. Familiarity with Mycoplasma-induced entities is important and can be useful in dermatological practice in determining the etiology and implementing appropriate treatment.

Abstract Purpose To summarize the outcomes of corneal sight rehabilitating surgery in Stevens-Johnson syndrome (SJS). Methods This is a retrospective analysis of a consecutive case series. Twenty-four eyes of 18 SJS patients were included in this study. The ocular parameters, surgical procedures, postoperative complications, and additional treatments of the cases were reviewed. Results A total of 29 corneal sight rehabilitating surgeries, which consists of 9 keratoplasties, 8 Keratolimbal allograft (KLAL) and 12 combined surgeries (keratoplasty and KLAL simultaneously) were performed on the 24 eyes. All patients were treated with glucocorticoid eyedrops and tacrolimus eyedrops for anti-rejection treatment without combining systemic immunosuppression, except two patients who were prescribed prednisone tablets for the management of systemic conditions. The mean follow-up period was 50.6 ± 28.1 months. The optimal visual acuity (VA) (0.74 ± 0.60 logarithm of the minimum angle of resolution [logMAR]) and endpoint VA (1.06 ± 0.82 logMAR) were both significantly better than the preoperative VA (1.96 ± 0.43 logMAR) (95% CI, p = 0.000). 57.1% patients (8/14) were no longer in the low vision spectrum, and 88.9% patients (8/9) were no longer blind. The mean epithelialization time was 7.1 ± 7.6 weeks. The success rate was 86.7%. Additional treatments for improving epithelialization included administration of serum eyedrops (n = 10), contact lens (n = 15), amniotic membrane transplantation (n = 6), and tarsorrhaphy (n = 8). Complications included delayed epithelialization (n = 4, over 12 weeks), glaucoma (n = 11), and severe allograft opacity (n = 4). Only one graft rejection was observed. Conclusions Keratoplasty and KLAL can remarkably enhance VA and improve low vision or even eliminate blindness for ocular complications of SJS. The outcome of the surgeries was correlated with the preoperative ocular situation and choice of operative methods.

Abstract Introduction Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening acute mucocutaneous disorders usually triggered by drugs. In this study, we aimed to evaluate the factors affecting mortality in patients with SJS-TEN. Methods Our study is a retrospective cohort study, analyzing data collected from a total of 12 tertiary care centers between April 2012 and April 2022. Results The study included 59 males and 107 females, a total of 166 patients, with an average age of 50.91 ± 21.25 years. Disease classification was TEN in 50% of cases, SJS in 33.1%, and SJS-TEN overlap in 16.9%. The average SCORTEN within the first 24 h was 2.44 ± 1.42. Supportive care was provided to 99.4% of patients. The most commonly used systemic immunomodulatory treatments were systemic steroids (84.3%), IVIG (intravenous immunoglobulin) (49.3%), and cyclosporine (38.6%). Plasmapheresis was administered to five patients. While 66.3% of patients were discharged, 24.1% resulted in exitus. Our comparative analysis of survivors and deceased patients found no effect of systemic steroids, IVIG, and cyclosporine treatments on mortality. Univariate analysis revealed that the SCORTEN scores on days 1 and 3 as well as the rates of detachment at the onset and during follow-up were significantly higher in deceased patients compared to survivors. The rates of fever, positive blood cultures, and systemic antibiotic use were higher in deceased patients compared to survivors. The presence of comorbidities, diabetes, and malignancy were significantly more common in deceased patients. Multivariate regression analysis indicated that over SCORTEN 2, the mortality risk exponentially rose with each SCORTEN increment, culminating in an 84-fold increase in mortality at SCORTEN 5–6 (odds ratio [95% confidence interval]: 13.902–507.537, p

IntroductionImmune-related epidermal necrolysis (irEN), including Stevens-Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN), represents a potentially lethal reaction to immune checkpoint inhibitors. An optimal treatment strategy remains undefined. This study evaluates the effectiveness and safety of combination therapy with corticosteroids and tumor necrosis factor inhibitors (TNFi) in treating irEN patients.MethodsIn this single-center, prospective, observational study, patients with irEN received either corticosteroid monotherapy or a combination therapy of corticosteroids and TNFi (etanercept for SJS, infliximab for TEN). The primary endpoint was re-epithelization time, with secondary endpoints including corticosteroid exposure, major adverse event incidence, acute mortality rates, and biomarkers indicating disease activity and prognosis. The study was registered at the Chinese Clinical Trial Registry (ChiCTR2100051052).ResultsThirty-two patients were enrolled (21 SJS, 11 TEN); 14 received combination therapy and 18 received corticosteroid monotherapy. IrEN typically occurred after 1 cycle of ICI administration, with a median latency of 16 days. Despite higher SCORTEN scores in the combination group (3 vs. 2, p = 0.008), these patients experienced faster re-epithelization (14 vs. 21 days; p < 0.001), shorter corticosteroid treatment duration (22 vs. 32 days; p = 0.005), and lower prednisone cumulative dose (1177 mg vs. 1594 mg; p = 0.073). Major adverse event rates were similar between groups. Three deaths occurred due to lung infection or disseminated intravascular coagulation, with mortality rates for both groups lower than predicted. Potential risk factors for increased mortality included continuous reduction in lymphocyte subset counts (CD4+ T cells, CD8+ T cells, natural killer cells) and consistent rises in inflammatory markers (serum ferritin, interleukin-6, TNF-α). Re-epithelization time negatively correlated with body mass index and positively correlated with epidermal detachment area and serum levels of interleukin-6 and TNF-α.ConclusionsCorticosteroids combined with TNFi markedly promote re-epithelization, reduce corticosteroid use, and decrease acute mortality in irEN patients without increasing major adverse events, offering a superior alternative to corticosteroid monotherapy. Inflammatory markers and lymphocyte subsets are valuable for assessing disease activity and prognosis.

Background/AimTo evaluate differences in ocular complications of Stevens Johnson Syndrome (SJS)/Toxic Epidermal Necrolysis (TEN) patients receiving either systemic IVIG or Ciclosporin (CsA) as initial treatments.MethodsRetrospective review of consecutive patients admitted for SJS/TEN at the Singapore General Hospital (SGH) from 2011 to 2017 who received either IVIG or Ciclosporin at the onset of the disease and had ophthalmological follow-up of at least 6 months were included. Acute ocular severity of SJS/TEN was graded using the Gregory grading score; chronic ocular complications were graded using the Sotozono system.ResultsA total of 18 subjects were included for analysis, with eight in the IVIG group and 10 in the CsA group. There were no significant differences in acute Gregory severity grading between the two groups. The CsA group had a trend towards worse overall chronic Sotozono grading scores compared to the IVIG group (median [IQR]: 2 [0–3] vs. 1 [0–6.5], p = 0.27), with a higher incidence of acute severe cornea involvement (60% vs. 25%, p = 0.93) and chronic corneal and eyelid involvement in the former than the latter. SJS/TEN patients with worse acute ocular involvement were more likely to have TEN and perianal mucosal involvement (50% vs. 0, p = 0.01).ConclusionCompared to those who received IVIG, SJS/TEN patients who received CsA at the acute disease stage, seemed to have worse acute corneal and chronic corneal and eyelid complications. Larger studies are needed to confirm this finding.

Background: Severe cutaneous adverse reactions (SCAR) are a group of delayed presumed T-cell mediated hypersensitivities associated with significant morbidity and mortality. Despite their shared global healthcare burden and impact, the clinical phenotypes, genomic predisposition, drug causality, and treatment outcomes may vary. We describe the establishment and results from the first Australasian registry for SCAR (AUS-SCAR), that via a collaborative network advances strategies for the prevention, diagnosis and treatment of SCAR. Methods: Prospective multi-center registry of SCAR in Australian adult and adolescents, with planned regional expansion. The registry collects externally verified phenotypic data drug causality, therapeutics and long-term patient outcomes. In addition, biorepository specimens and DNA are collected at participating sites. Results: we report on the first 100 patients enrolled in the AUS-SCAR database. DRESS (50%) is the most predominant phenotype followed by SJS/TEN (39%) and AGEP (10%), with median age of 52 years old (IQR 37.5, 66) with 1:1 male-to-female ratio. The median latency for all implicated drugs is highly variable but similar for DRESS (median 15 days IQR 5,25) and SJS/TEN (median 21 days, IQR 7,27), while lowest for AGEP (median 2.5 days, IQR 1,8). Antibiotics (54.5%) are more commonly listed as primary implicated drug compare with non-antibiotics agent (45.5%). Mortality rate at 90 days was highest in SJS/TEN at 23.1%, followed by DRESS (4%) and AGEP (0%). Conclusion: In the first prospective national phenotypic and biorepository of SCAR in the southern hemisphere we demonstrate notable differences to other reported registries; including DRESS-predominant phenotype, varied antibiotic causality and low overall mortality rate. This study also highlights the lack of standardised preventative pharmacogenomic measures and in vitro/in vivo diagnostic strategies to ascertain drug causality. Trial registration: ANZCTR ACTRN12619000241134. Registered 19 February 2019.

Stevens-Johnson Syndrome (SJS) is a severe and rare adverse drug reaction associated with significant morbidity and mortality. Although SJS is commonly triggered by multiple drugs, non-steroidal anti-inflammatory drugs (NSAIDs), including diclofenac, have been frequently implicated. A middle-aged woman, who is 50 years old, has a prior medical record of high blood pressure, type 2 diabetes, and has recently suffered from a pulmonary embolism. She was later admitted to the intensive care unit (ICU), where she was ultimately diagnosed with Steven Johnson syndrome. Careful drug selection, close monitoring of patients with predisposing factors, and prompt identification of adverse events are crucial to prevent severe drug reactions.

Quetiapine, a second-generation antipsychotic drug, commonly has very less propensity of extrapyramidal symptoms and has an added advantage of less risk of serum prolactin, weight gain, or metabolic syndrome than other second-generation antipsychotics. Stevens–Johnson syndrome (SJS) is more commonly reported with anticonvulsant use, and less commonly with antipsychotics. To our knowledge, there is very few evidence of quetiapine-induced SJS in literature. Hence, we present a case of SJS in a patient who was on quetiapine.

Min Lin,1,&ast; Ting Gong,2,&ast; Shifan Ruan,1,&ast; Xiaoqing Lv,1 Rongying Chen,1 Xinhong Su,1 Bo Cheng,1 Chao Ji1 1Department of Dermatology, The First Affiliated Hospital of Fujian Medical University, Fuzhou, 350000, People’s Republic of China; 2Department of Central Laboratory, The First Affiliated Hospital of Fujian Medical University, Fuzhou, 350000, People’s Republic of China&ast;These authors contributed equally to this workCorrespondence: Bo Cheng; Chao Ji, Department of Dermatology, The First Affiliated Hospital of Fujian Medical University, 20 Chazhong Road, Taijiang District, Fuzhou, 350000, People’s Republic of China, Tel +86 13859024296 ; +86 18651619908, Email bochengg@163.com; jichaofy@fjmu.edu.cnObjective: Anticancer drugs have revolutionized tumor therapy, with cutaneous toxicities such as Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) being common immune-related adverse events. The debate over the efficacy of systemic corticosteroids in treating these conditions persists, while tumor necrosis factor (TNF)-alpha inhibitors show promise. This study aims to evaluate the effectiveness and safety of combination therapy involving the TNF-α inhibitor adalimumab for SJS/TEN induced by anticancer drugs.Methods: A literature review of SJS/TEN cases induced by anticancer drugs from 1992 to 2023 was conducted, alongside an analysis of patients admitted to the First Affiliated Hospital of Fujian Medical University during the same period. Clinical characteristics, skin healing time, mortality, and adverse events were evaluated in two treatment groups: SJS/TEN patients treated with targeted anticancer therapies and immunotherapies.Results: Among the 27 patients studied (18 with SJS or SJS-TEN overlapping and 9 with TEN), combination therapy with adalimumab significantly reduced mucocutaneous reepithelization time and healing duration compared to corticosteroid monotherapy. Patients receiving adalimumab combined with corticosteroids had lower actual mortality rates than those on corticosteroid monotherapy. The combination therapy also showed a trend towards reducing standardized mortality rates based on the Score of Toxic Epidermal Necrolysis (SCORTEN).Conclusion: The findings suggest that adalimumab in combination with corticosteroids provides significant clinical benefits and is safer than corticosteroids alone for treating SJS/TEN induced by targeted anticancer therapies and immunotherapies. This study contributes valuable insights into potential treatment strategies for severe cutaneous adverse reactions to anticancer drugs, highlighting the importance of exploring alternative therapies such as TNF-α inhibitors in managing these conditions effectively.Keywords: anti-TNF-α, adalimumab, treatment, Stevens-Johnson syndrome, toxic epidermal necrolysis

Stevens-Johnson Syndrome is considered a life-threatening adverse drug reaction. The pathogenesis of these syndromesis still unclear, but several drugs, such as anticonvulsivants and antibiotics, and especially sulfonamides, non-steroidal anti-infiammatorydrugs, and allopurinol were predominantly suspected of triggering this reaction. A 5-year-old boy patient who came to hospital attention for an urticarial reaction developed after taking amoxicillin, then a recent scarlet fever acquired by brother. Due to the worsening of the lesions, he was admitted to our PICU after being intubated for deterioration of the respiratory dynamics and safe treatment of secretions. Nursing care is crucial: care of patient hospitalized with Stevens-Johnson syndrome and Toxic Epidermal Necrolysis consists of wound care, infection prevention, comfort management, hydration and nutrition, psychosocial support, and prevention of long-term complications. For all patients with SJS and TEN, it is essential to perform a total body daily evaluation of the skin. If the dressings remain intact, it is advisable to note the appearance of visible skin and any visible exudate or staining on the outside of the intact dressings. If dressings are being removed or need to be reapplied daily, a full skin evaluation is useful.

Abstract Introduction This study analysed the causative factors and clinical characteristics of acute and chronic ocular sequelae of Stevens–Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) treated at a large third-referral centre in a developed country (Hungary) over a 15-year period. Methods This was a retrospective review of patients with acute and/or chronic SJS/TEN who were managed between 2006 and 2020 at the Department of Ophthalmology of Semmelweis University in Budapest, Hungary. For each subject, clinical data, including patient demographics, clinical history, causative agents of SJS/TEN, and conservative and surgical treatment details, were reviewed. Results Ninety-six eyes of 48 patients were included (28 female; 58.3%); the age at disease onset was 32.1 ± 22.4 years. The most common causative factors were medicines (n = 36; 75.0%). Among these drugs, 29.2% were nonsteroidal anti-inflammatory drugs (NSAIDs) (n = 14), 20.8% were antibiotics (n = 10) and 14.6% were antiepileptic drugs (n = 7). In patients with chronic SJS/TEN, the most commonly found ocular sequelae were conjunctival hyperaemia in 45 (56.3%) eyes, symblepharon in 38 (47.5%) eyes, trichiasis/distichiasis in 37 (46.3%) eyes, corneal neovascularization in 31 (38.8%) eyes and corneal scarring in 29 (36.3%) eyes. In patients with chronic SJS/TEN, the most frequently used topical conservative treatment included antibiotics in 53 (66.3%) eyes, preservative-free artificial tears in 50 (62.5%) eyes and topical corticosteroids in 42 (52.5%) eyes of 40 patients. The most frequently performed ocular surgeries for managing chronic ocular sequelae in patients with SJS/TEN were epilation for trichiasis (n = 27; 33.8%), cataract surgery (n = 14; 17.5%), entropion surgery (n = 12; 15.0%), penetrating keratoplasty (PK) (n = 11; 13.8%) and amniotic membrane transplantation (n = 4; 5.0%). Conclusion Our results suggest that NSAIDs, antibiotics and antiepileptic drugs are the most common causative factors for SJS/TEN in Hungary. Like in other countries, in Hungary, the ocular management of patients with acute and chronic SJS/TEN is heterogeneous, and most cases do not follow modern therapeutic guidelines.

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.

Background: Fixed drug eruption (FDE) is a type of drug-induced skin inflammation characterized by the recurrence of lesions in the same region following repeated exposure to the causative drug. FDE typically presents as localized spots or plaques without systemic symptoms; however, it can manifest in other forms, such as blisters and papules. In FDE, effector memory CD8-positive T cells that remain dormant in the basal layer after a previous inflammation are reactivated upon re-exposure to the causative drug, leading to the development of erythema at the same sites. Case Presentation: Herein, we report the case of a 23-year-old man who developed ibuprofen-induced multiple FDE. The diagnosis was confirmed by detecting a rash immediately following ibuprofen administration, and histopathological findings were consistent with FDE. Ibuprofen is widely available as an over-the-counter medication, and patients may not always report its use—making the diagnosis of ibuprofen-induced FDE particularly challenging. Approximately 24 h following drug-induced CD8-positive T cell activation, regulatory T cells normally infiltrate the epidermis to suppress inflammation and promote resolution. However, in multiple FDE, CD8-positive T cell activity may outweigh that of regulatory T cells, causing uncontrolled inflammation and leading to the spread of poorly-demarcated lesions that can progress to severe drug reactions such as Stevens–Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN). We reviewed 13 cases of ibuprofen-induced multiple FDE. Conclusions: Over-the-counter medications can cause multiple FDEs, and the repeated administration of the causative drug can result in severe reactions such as SJS/TEN. The early diagnosis and strict discontinuation of the causative drugs are therefore crucial.

IntroductionStevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare yet life-threatening adverse events associated with immune checkpoint inhibitors (ICIs). This systematic review synthesizes the current literature to elucidate the clinical characteristics and outcomes of patients with ICI-related SJS/TEN.MethodsWe conducted a thorough search across databases including Embase, Web of Science, Cochrane, MEDLINE, Scopus, and PubMed. Selection criteria focused on reports of SJS/TEN among cancer patients treated with ICIs, analyzing clinical manifestations, therapeutic interventions, and outcomes.ResultsOur analysis included 47 articles involving 50 patients with ICI-related SJS/TEN. The cohort had a mean age of 63 years, with a slight male predominance (54%). Most patients had melanoma or non-small cell lung cancer. SJS/TEN typically occurred early, with a median onset of 23 days post-ICI initiation. Treatment primarily involved systemic corticosteroids and intravenous immunoglobulins. The overall mortality rate was 20%, higher for TEN at 32%, with infections and tumor progression as leading causes. Median time from onset to death was 28 days. Survivors experienced a median re-epithelization time of 30 days, positively correlated with the extent of epidermal detachment (rs = 0.639, p = 0.009). Deceased patients exhibited a significantly higher proportion of TEN (90% vs. 48%, p = 0.029) and a larger epidermal detachment area (90% vs. 30% of the body surface area [BSA], p = 0.005) compared to survivors. The combination therapy group showed a higher proportion of TEN compared to corticosteroid monotherapy or non-corticosteroid therapy groups (72% vs. 29% and 50%, p = 0.01), with no significant differences in mortality or re-epithelization time. Dual ICI therapy resulted in a higher TEN rate than single therapy (100% vs. 50%, p = 0.028). Among single ICI therapies, the sintilimab-treated group trended towards a higher TEN rate (75% vs. 40-50%, p = 0.417), a larger detachment area (90% vs. 30-48% of BSA, p = 0.172), and a longer re-epithelization time (44 vs. 14-28 days, p = 0.036) compared to other ICI groups, while mortality rates remained similar.ConclusionICI-related SJS/TEN substantially impacts patient outcomes. Prospective clinical trials are critically needed to further clarify the pathogenesis and optimize therapeutic regimens.

Background: Antibiotics are the most commonly used drugs to treat bacterial infections; however, these life-saving drugs can also cause adverse drug reactions. In several pharmacovigilance studies, adverse drug reactions are a cause of prolonged hospitalization. The development of hypersensitivity reactions even after a negative skin test and patch test makes antibiotic-associated adverse reactions unpredictable. Owing to the scarcity of data on antibiotic desensitization, these drugs are important for pharmacovigilance. Most of the previous studies conducted were retrospective and lacked data regarding the duration of adverse drug reactions. Aim: This study aimed to examine adverse drug reactions associated with antibiotics and to provide knowledge on the duration of these reactions. Method: A prospective observational study was conducted on patients receiving antibiotics at a tertiary care hospital. The patients were followed until discharge, and causality and severity assessments were performed. Descriptive statistical analyses were performed to summarize the data. Results: The study results show the median duration of adverse drug reactions was two days, with an interquartile range of two to four days. Most (21,3%) of the reactions were caused by cephalosporins. The majority (44.7%) of the reactions were skin and subcutaneous tissue disorders. The majority (78.7%) of the reactions were moderately severe and were not responsible for prolonged hospital stay. Conclusion: The patients receiving antibiotics require close monitoring for early detection and management of adverse reactions to reduce the duration of hospital stay and the duration of adverse drug reactions.

AimTo evaluate the prevalence and clinical characteristics of limbal stem cell deficiency (LSCD) in the setting of a tertiary referral cornea practice at an academic center.Patient and methodsA retrospective chart review was performed to identify all unique medical record numbers (MRNs) presenting to a single cornea specialist (JHH) at the University of Minnesota during calendar years 2019 and 2020. Records were queried and confirmed for a diagnosis of LSCD. Clinical characteristics of identified patients, including demographics, etiology of LSCD, severity of LSCD, treatment, and best corrected visual acuity (BCVA) at final follow-up, were documented.ResultsIn total 1436 unique MRNs were identified over the study period. There were 61 individuals (91 eyes) diagnosed with LSCD, resulting in a prevalence of 4.25% (95% CI, 3.33-5.42). Of 91 eyes, 60 eyes were bilateral (65.9%). Among all eyes, ocular surface burns were the most common etiology (18.7%) followed by iatrogenic or medicamentosa (15.4%). There were 51 eyes (56.0%) that underwent some form of transplantation. The median BCVA at final follow-up was Snellen 20/80 (range 20/20 to no light perception).ConclusionsThe prevalence of LSCD found at a cornea subspecialty tertiary referral center in our study was much higher than previously reported prevalence rates. This may reflect referral bias and potential underdiagnosis of LSCD in practices outside of subspecialty referral centers. The high prevalence rate in our study also suggests that LSCD patients are concentrated in subspecialty referral practices, with many having high morbidity disease. This constitutes a major health burden for these practices.

Introduction: The paediatric population is prone to developing cutaneous adverse drug reactions. However, the incidence of Severe Cutaneous Adverse Drug Reactions (SCAR) is rare in this age group, with few studies describing such reactions in detail. Aim: To describe the clinico-epidemiological factors, drug profile, laboratory parameters, and treatment outcomes of SCAR in children admitted to a tertiary care centre in South India. Materials and Methods: A retrospective cohort study was carried out over a 10-year period, including paediatric patients (0-18 years) admitted to Dermatology, Medicine, and Paediatric wards in the tertiary care centre. Demographic details, suspected drugs, comorbidities, personal and family history of drug reactions, physical examination, laboratory parameters, treatment received along with its duration, and the state of morbidity and mortality were recorded. SPSS version 18.0 was used for analysis. Descriptive statistics were used to summarise the demographics and clinical characteristics of the patients. Results: Among all the patients admitted with SCAR, 27 (15%) belonged to the paediatric age group. The median age was 15 years, and the female-to-male ratio was 1.25. Nineteen (70.3%) were diagnosed with Stevens-Johnson Syndrome (SJS)/Toxic Epidermal Necrolysis (TEN), and eight (29.6%) were diagnosed with Drug Reaction with Eosinophilia and Systemic Symptom (DRESS). There were no cases of Acute Generalised Exanthematous Pustulosis (AGEP). The most common class of drugs implicated was antiepileptics (62.8%). Two patients (7%) had a family history of drug reactions. All patients had mucosal involvement. The majority of the children responded to intravenous steroids, and two required additional intravenous immunoglobulin injections for clinical improvement. All cases were cured with no mortality or long-term sequelae. Conclusion: The incidence of SCAR in the paediatric age group is significant. Anticonvulsants, particularly phenytoin, carbamazepine, and lamotrigine, need to be used with caution in this age group. Prompt diagnosis and treatment with systemic steroids can reduce mortality, morbidity, and long-term sequelae.

Background: Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and SJS-TEN overlap syndrome are rare severe cutaneous adverse reactions associated with high mortality. Objectives: To estimate incidence and describe trends of SJS/TEN hospitalizations in the United States and to describe the clinical, demographic, and geographic characteristics of affected patients and risk factors for mortality. Methods: We utilized hospitalization data from the 2010 to 2020 National Inpatient Sample. SJS, SJS-TEN overlap syndrome, and TEN were identified by International Classification of Diseases, 9th Revision and International Classification of Diseases, 10th Revision codes and analyzed by logistic regression. Results: We identified 51,040 hospitalizations involving SJS/TEN. Amog those, 37,283 (73.0%) were for SJS only, 7818 (15.3%) were for SJS-TEN overlap syndrome, and 7160 (14.0%) were for TEN only. Overall, SJS/TEN hospitalization rates declined over time, 2010 to 2020 (P

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) can occur at any age and are commonly caused by adverse drug events. Rapid diagnosis of SJS/TEN is imperative, followed by immediate cessation of offending agent and induction of appropriate treatment. Cyclosporine, a calcineurin inhibitor, has been reported to have a promising therapeutic effect in SJS/TEN patients with few side effects. Diagnosis of SJS/TEN in children is especially challenging as many of the symptoms mimic that of an upper respiratory infection, or other viral entities such as cocksackie A, roseola, or herpes simplex virus. We recommend initiating cyclosporine modified treatment, especially in children, upon any suspicion of SJS/TEN in a patient in order to halt the disease progression as early as possible.

Takla R Anis,1 John Meher2 1Pharmacy Department, Henry Mayo Newhall Hospital, Valencia, CA, USA; 2Emergency Department, Henry Mayo Newhall Hospital, Valencia, CA, USACorrespondence: Takla R Anis, Email Takla.anis@gmail.comAbstract: Allopurinol is a commonly used medication that lowers uric acid production which is essential for gout treatment and prevention. Although many patients tolerate allopurinol therapy without severe complications; Stevens–Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are life-threatening delayed hypersensitivity reactions that have been reported especially among Asian and African American patients. We describe a case of allopurinol-induced SJS in a 95-year-old Asian female. The patient started allopurinol 13 days prior to presenting to the emergency room (ER). On day 10 of therapy, the patient developed a diffuse erythematous desquamating rash which prompted her to visit the ER after 3 days from the rash onset. This case report describes a rare fatal hypersensitivity reaction that requires rapid identification and treatment in a multi-disciplinary setting.Keywords: Stevens–Johnson syndrome, allopurinol, genetic testing, hypersensitivity

Stevens-Johnson syndrome (SJS) is a life-threatening mucocutaneous disease with various etiologies including drugs, infections, and malignancies. Ocular manifestations of SJS vary from the membrane, symblepharon formation, and epithelial defect in the acute phase to trichiasis, eyelid margin keratinization, and lacrimal duct obstruction in the chronic phase. A 13-year-old boy with a history of drug-induced SJS presented to our clinic complaining of a mass in the nasal side and inferior fornix of the right eye from 1 year ago. The mass-like lesion in the medial side of the right eye was accompanied by ankyloblepharon, symblepharon, and ptosis and limited ocular movement. Orbital imaging showed cystic lesions on the medial side of the right globe and the inferior fornix. Two large cysts were entirely surgically excised. Histopathologic investigation revealed conjunctival tissue with nonkeratinized epithelium and goblet cells. There was no sign of conjunctival cyst recurrence or symblepharon formation on the 6th-month follow-up. The inferior fornix achieved acceptable depth and the ocular movements became normal.

Weiqiang Fei,1 Jun Shen,2 Hui Cai3 1College of Nursing, Hangzhou Vocational & Technical College, Hangzhou, Zhejiang Province, People’s Republic of China; 2Nursing Department, Sir Run Run Shaw Hospital Affiliated to Zhejiang University School of Medicine, Hangzhou, Zhejiang Province, People’s Republic of China; 3Nursing Department, Jiangsu Province Hospital Chinese Medicine, Affiliated Hospital of Nanjing University of Chinese Medicine, Nanjing, People’s Republic of ChinaCorrespondence: Weiqiang Fei, College of Nursing, Hangzhou Vocational & Technical College, 68 Xueyuan Street, Xiasha Higher Education Park, Qiantang District, Hangzhou, Zhejiang Province, 310018, People’s Republic of China, Tel/Fax +86-571-56700120, Email weiqiangfei@126.comPurpose: The purpose of the study is to analyze FAERS data to identify drugs associated with Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), determine demographics, drug classes involved, most likely resulted in death, and highlight emerging trends in SJS/TEN reactions.Patients and Methods: We reviewed the publicly available FAERS database from 2004– 2021. Using search terms “Stevens-Johnson syndrome” or “Toxic epidermal necrolysis”, we identified the reports of SJS/TEN or SJS/TEN followed by death that might associated with specific drugs. Then the amounts and trends were counted analyzed.Results: During the study period of 2004– 2021, the Food and Drug Administration (FDA) received a total of 14,363,139 reports of adverse reactions, among which 24,976 were linked to SJS or TEN. After excluding the cases with incomplete or insufficient information on age, gender, or country of origin, the median median age of patients was 53.82 (IQR = 57.52), the females accounted for 56.59% (12,827 cases) and 8,507 (38.34%) originated in the United States. The top 50 drugs were associated with 15,149 cases (60.65%). The subsequent fatal outcome occurring in 4878 out of 24,976 cases (19.53%). Top 3 drug classes associated with SJS/TEN in FAERS were antiepileptics, non-steroidal anti-inflammatory drugs (NSAIDs) and others. Top drug classes associated with SJS/TEN deaths were antineoplastic agents and cephalosporins. Linear regression showed that the annual percentage of monoclonal antibody-related SJS/TEN reactions increased at an average rate of 0.25% (95% confidence interval: 0.18, 0.32) from 0.00% in 2004 to 4.79% in 2021, faster than any other drug class except antigout drug (allopurinol).Conclusion: By using the publicly available FAERS data, we have identified some important themes and trends in drug-related SJS/TEN reactions. Monoclonal antibodies and proton pump inhibitors are drugs with emerging trends causing SJS/TEN. Additionally, cephalosporin antibiotics have a higher mortality rate following SJS/TEN.Keywords: Stevens-Johnson syndrome, SJS, toxic epidermal necrolysis, TEN, adverse drug reactions, fatal outcome, trends

Imiquimod 5% cream is an approved treatment for actinic keratoses, superficial basal cell carcinomas and anogenital warts. Severe systemic side effects associated to imiquimod 5% cream are rare, although few cases of erythema multiforme and Stevens-Johnson syndrome have been described. We present a case of Stevens-Johnson syndrome associated to topical treatment with imiquimod of two superficial basal cell carcinomas.

Abstract Background and Aims Adverse cutaneous drug reactions (ACDRs) are common and potentially life‐threatening, while also hindering patient compliance to medications. Given the regional differences in patterns and prevalence of ACDRs, it is important to study the epidemiology, as well as the clinical and outcome patterns of patients with ACDRs in Iran. Methods This cross‐sectional study on ACDRs was conducted among hospitalized patients in a referral university hospital in the city of Isfahan, Iran. The patients' demographics, clinical information, and outcomes, including age, gender, past medical history, medication history, drug reaction with eosinophilia and systemic symptoms (DRESS) diagnosis, Steven‐Johnson Syndrome (SJS) diagnosis, toxic epidermal necrosis (TEN) diagnosis, treatment regimen (steroids or intravenous immunoglobulin [IVIG]) and outcome information, including intensive care requirements, severe medical complications, or death, were obtained from medical records. Results A total of 195 patients with a mean age of 40 years and consisting of 61% females were included. Carbamazepine, lamotrigine, sodium valproate, and phenytoin are the most commonly reported medications. Rate of complications was 45% with DRESS, SJS, and TEN diagnosed in 26%, 47%, and 19%, respectively. Treatment was carried out with steroids and IVIG in 81% and 19%, respectively. Among patients, 15% required intensive care and 5% died. Diagnosis of TEN, older age, and baseline heart disease were predictors of mortality. Patients with SJS were younger and more likely to be males, and they were more likely to have eye complications. On the other hand, patients with the diagnosis of TEN were more likely to receive IVIG and intensive care, and had a higher mortality rate. Conclusion Our study provides insight into the demographics and clinical patterns of Iranian patients with ACDRs. This will help in predicting rates of complications, treatments, and outcomes in patients and therefore make proper management decisions.

Abstract Background Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare and extremely serious drug-induced dermatological disorders. The ocular surface condition at the early stage has been little studied and should contribute to novel perspectives in early and effective topical therapy of these diseases. The objectives of the study were to evaluate the acute phase of ocular surface involvement and histopathologic changes in patients with acute SJS/TEN. Methods Ten patients with acute phase of SJS/TEN onset and eleven age- and sex-matched healthy volunteers were recruited. Ocular surface symptoms and signs, conjunctival impression cytology, and tear multi-cytokine were assessed. Results Ocular surface objective signs were normal at the acute stage of SJS/TEN, while most patients have abnormal ocular surface subjective symptoms and meibomian gland secretion. Conjunctival impression cytology showed a significant decrease in goblet cell density and severe ocular surface squamous metaplasia in acute SJS/TEN patients. Tear multi-cytokine analysis showed all 21 pro- and anti-inflammatory cytokines all sharply elevated. Goblet cell density was significantly negatively correlated with tear C-X3-C motif chemokine ligand 1 (CX3CL1) and interleukin 13. Conclusions Severe pathologic squamous metaplasia and inflammation onset in the ocular surface at the acute stage of the SJS/TEN, even if the ocular surface condition seemed basically normal with adequate systemic immunosuppressant and general supportive treatment. Early topical anti-inflammatory therapy should be carried out actively.

Abstract Background Ultraviolet radiation (UVR) exposure is commonly reported as a risk factor for Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). However, minimal evaluation of photo-induced SJS/TEN has been conducted. Thus, this review identifies all cases of SJS/TEN that are linked to an acute exposure of UVR and outlines the unifying characteristics of these cases. Furthermore, the theoretical pathogenesis, differential diagnoses, and proposed diagnostic criteria are defined. Methods PubMed, Google Scholar, and other databases and websites were searched from inception to September 2021 to identify studies that met inclusion criteria. The following keywords were utilized: “Stevens-Johnson syndrome” and “toxic epidermal necrolysis” with “ultraviolet,” “photodistributed,” “photo-induced,” “photosensitivity,” and “photo.” One reviewer assessed study characteristics, with confirmation by a second. The risk of bias was assessed independently by another. Results Thirteen patient cases were identified, all reporting ultraviolet radiation prior to rash onset and an underlying causal drug. Case classifications included 7/13 SJS and 6/13 TEN. All cases described the rash as photodistributed with UVR exposure prior to rash onset (delay of 1–3 days) and a causal drug. 10 cases provided evidence that the photodistributed rash lacked linear demarcation (as in a sunburn) with satellite target-like lesions. No cases described a flu-like prodrome. Discussion Mucositis, palmar and plantar rash, a positive Nikolsky sign, and a prolonged disease course can help distinguish from photosensitive reactions, while a negative direct immunofluorescence test is important to distinguish from other photo-induced disorders. Conclusion Physicians should be aware that UVR may precipitate SJS/TEN in patients taking susceptible drugs. After a 24-h delay from UVR exposure, a non-distinct, photodistributed rash appears with no flu-like prodrome and progresses for at least 48 h to include vesiculobullous eruptions and mucous membrane involvement. Photodistributed SJS/TEN appears to be photo-drug-induced with a unique onset and rash presentation that should be recognized as a distinct diagnosis.

Abstract Introduction Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe cutaneous adverse reactions with high morbidity and mortality and not clearly established treatment protocol. This meta-analysis aimed to evaluate the efficacy and safety of three biologic TNF-α inhibitors (infliximab, etanercept, adalimumab) in the treatment of SJS, SJS-TEN overlap, and TEN. Methods Electronic databases were searched for original studies containing human participants diagnosed with SJS/TEN and treated with biologic TNF-α inhibitors. Individual patient data were collected and summarized to provide a comprehensive overview on therapeutic efficacy of different biologic TNF-α inhibitors for SJS, SJS-TEN overlap, and TEN, respectively. Meta-analyses on aggregated study data were conducted using random-effects model. Results Overall, 55 studies with 125 sets of individual patient data were included. Infliximab was used to treat 3 patients with SJS-TEN overlap and 28 patients with TEN, and the actual mortality rate was 33.3% and 17%, respectively. Etanercept was administered to 17 patients with SJS, 9 patients with SJS-TEN overlap, and 64 patients with TEN, and mortality rate was reported to be 0%, 0%, and 12.5%, respectively. For participants with TEN, no significant difference was found in time of reepithelialization, hospitalization time, and mortality rate comparing etanercept with infliximab. More sequelae were reported in patients receiving infliximab than in patients treated with etanercept (39.3% versus 6.4%). Adalimumab was administered to four patients with TEN, and mortality rate was 25%. Meta-analyses on aggregated study data revealed significantly shortened hospitalization time in etanercept compared with non-etanercept groups [weighted mean differences (WMD) −5.30; 95% confidence interval (CI) −8.65 to −1.96]. Etanercept was associated with a survival benefit for patients when compared with non-etanercept treatment, however, the analysis was not statistically significant (odds ratio 0.55; 95% CI 0.23–1.33). Conclusions On the basis of the current findings, etanercept is currently the most promising biologic therapy for SJS/TEN. Further evaluation in prospective studies is required to confirm its efficacy and safety.

Abstract The aim of our review article was to summarize the current literature on Stevens–Johnson syndrome (SJS) and its severe form, toxic epidermal necrolysis (TEN). SJS/TEN is a serious, rare multi-system, immune-mediated, mucocutaneous disease with a significant mortality rate that can lead to severe ocular surface sequelae and even to bilateral blindness. Restoration of the ocular surface in acute and chronic SJS/TEN is challenging. There are only limited local or systemic treatment options for SJS/TEN. Early diagnosis, timely amniotic membrane transplantation and aggressive topical management in acute SJS/TEN are necessary to prevent long-term, chronic ocular complications. Although the primary aim of acute care is to save the life of the patient, ophthalmologists should regularly examine patients already in the acute phase, which should also be followed by systematic ophthalmic examination in the chronic phase. Herein, we summarize actual knowledge on the epidemiology, aetiology, pathology, clinical appearance and treatment of SJS/TEN.

Mycoplasma pneumoniae is a leading cause of a community-acquired respiratory illness occurring in children with manifestations occurring throughout the year but peaking in summer and early fall. Predominantly affecting school-aged children, the infection presents as pneumonia, featuring fever, cough, dyspnea, and sore throat. Extrapulmonary manifestations such as Stevens-Johnson have been rarely associated with mycoplasma pneumoniae infection presenting with ocular, oral, and genital involvement. We report a case of a 7-year-11-month-old girl presenting with a 9-day fever history and 3-day bilateral conjunctivitis, aphthous stomatitis, and mucositis. After several investigations, a diagnosis was made and results showed a mycoplasma pneumoniae infection complicated with Steven-Johnson syndrome.

Background: The treatment paradigm for advanced non-small-cell lung cancer (NSCLC) is rapidly changing. Epidermal growth factor receptor tyrosine kinase inhibitors (EGFR-TKIs) and anti-programmed death-1 (PD-1) antibodies have increasingly been incorporated into routine care for nearly all patients with NSCLC. Toripalimab was recently approved as the first-line treatment for advanced non-squamous NSCLC in combination with chemotherapy. Stevens–Johnson syndrome (SJS) is a rare but potentially fatal complication of TKI and anti-PD-1 therapy. We reported a case of SJS after sequential use of EGFR-TKIs and toripalimab in an NSCLC patient with EGFR mutations 19 del/T790M/C797S in trans and cis.Case presentation: A 58-year-old man with stage IV NSCLC received gefitinib because next-generation sequencing (NGS) revealed an EGFR 19del, followed by osimertinib and pemetrexed with the emergence of EGFR T790M. Four EGFR mutations 19 del/T790M/C797S in trans and cis were detected after osimertinib resistance. The combination of toripalimab and docetaxel was administered as a third-line treatment. The patient developed SJS at 21 days, and toripalimab was discontinued. After treatment with methylprednisolone and prednisolone, the skin toxicity of the patient gradually decreased and eventually disappeared. The patient received osimertinib and anlotinib after recovery, and SJS has not recurred. The ongoing treatment is still effective and results in stable disease.Conclusion: We reported the first case of SJS induced by toripalimab in a patient with lung adenocarcinoma harboring multiple EGFR mutations. The TKI treatment after SJS was well tolerated and effective.

Objective: To review the cases of Stevens-Johnson syndrome and/or toxic epidermal necrolysis in adult male patients to determine the incidence of genitourinary manifestations, the indication for urethral catheters, and to provide recommendations for management. Materials and methods: This is a retrospective observational study of adult male patients over a ten year period. The study group is divided into patients with and without genitourinary manifestations. Results: We identified 57 patients who met the study inclusion criteria, of whom 39 had genitourinary involvement. The most common location of lesions was the phallus although many patients had multiple sites of involvement. These lesions were treated similarly compared to other nongenitourinary cutaneous lesions. Four patients presented with dysuria, one with frequency, and one with hesitancy and intermittency. A urethral catheter was placed in 25 of the 39 patients. None of the patients who were not catheterized and did not have lower urinary tract symptoms at the time of presentation developed voiding symptoms during their hospital stay. Apart from a one-time episode of incontinence in one patient that resolved spontaneously, none of the patients who were catheterized developed voiding issues after their catheters were removed. No patients required follow-up with urology after discharge. Conclusions: No patients developed a symptomatic urethral stricture. Many patients had multiple sites of involvement. Despite no standardized treatment being used, all cutaneous lesions were successfully treated in patients who survived their illness, with documented resolution of genitourinary lesions on physical examination. Routine urethral catheterization and urologic consultation are not necessary in these patients.

Introduction Enfortumab vedotin is a novel drug for locally advanced or metastatic urothelial carcinoma, but it is associated with a high incidence of skin reactions (up to 47.0%). Case presentation A 71‐year‐old male was administered enfortumab vedotin for bladder cancer associated with lymph node metastases. Slight erythema of the upper limbs appeared on Day 5. Erythema gradually worsened. On Day 8, second administration was performed. On Day 12, based on the extents of blisters, erosion, and epidermolysis, a diagnosis of toxic epidermal necrolysis was made. The patient died of multiple organ failure on Day 18. Conclusion As serious cutaneous toxicity may appear early after the start of administration, it is important to consider the timing of the second administration of the initial course carefully. In cases of skin reaction, reduction or discontinuation should be considered.

Abstract Stevens-Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) are serious and rare diseases, most often drug-induced, and their incidence has been estimated at 6 cases/million/year in France. SJS and TEN belong to the same spectrum of disease known as epidermal necrolysis (EN). They are characterized by more or less extensive epidermal detachment, associated with mucous membrane involvement, and may be complicated during the acute phase by fatal multiorgan failure. SJS and TEN can lead to severe ophthalmologic sequelae. There are no recommendations for ocular management during the chronic phase. We conducted a national audit of current practice in the 11 sites of the French reference center for toxic bullous dermatoses and a review of the literature to establish therapeutic consensus guidelines. Ophthalmologists and dermatologists from the French reference center for epidermal necrolysis were asked to complete a questionnaire on management practices in the chronic phase of SJS/TEN. The survey focused on the presence of a referent ophthalmologist at the center, the use of local treatments (artificial tears, corticosteroid eye drops, antibiotic-corticosteroids, antiseptics, vitamin A ointment (VA), cyclosporine, tacrolimus), the management of trichiatic eyelashes, meibomian dysfunction, symblepharons, and corneal neovascularization, as well as the contactologic solutions implemented. Eleven ophthalmologists and 9 dermatologists from 9 of the 11 centers responded to the questionnaire. Based on questionnaire results, 10/11 ophthalmologists systematically prescribed preservative-free artificial tears, and 11/11 administered VA. Antiseptic or antibiotic eye drops or antibiotic-corticosteroid eye drops were recommended as needed by 8/11 and 7/11 ophthalmologists, respectively. In case of chronic inflammation, topical cyclosporine was consistently proposed by 11/11 ophthalmologists. The removal of trichiatic eyelashes was mainly performed by 10/11 ophthalmologists. Patients were referred to a reference center for fitting of scleral lenses (10/10,100%). Based on this practice audit and literature review, we propose an evaluation form to facilitate ophthalmic data collection in the chronic phase of EN and we also propose an algorithm for the ophthalmologic management of ocular sequelae.

Abstract Background Long-term sequelae are frequent and often disabling after epidermal necrolysis (Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN)). However, consensus on the modalities of management of these sequelae is lacking. Objectives We conducted an international multicentric DELPHI exercise to establish a multidisciplinary expert consensus to standardize recommendations regarding management of SJS/TEN sequelae. Methods Participants were sent a survey via the online tool “Survey Monkey” consisting of 54 statements organized into 8 topics: general recommendations, professionals involved, skin, oral mucosa and teeth, eyes, genital area, mental health, and allergy workup. Participants evaluated the level of appropriateness of each statement on a scale of 1 (extremely inappropriate) to 9 (extremely appropriate). Results were analyzed according to the RAND/UCLA Appropriateness Method. Results Fifty-two healthcare professionals participated. After the first round, a consensus was obtained for 100% of 54 initially proposed statements (disagreement index