Toxic Epidermal Necrolysis/Stevens-Johnson Syndrome at A University Hospital in Saudi: Causative Factors and Outcomes

Introduction:Toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS) are rare, life-threatening conditions caused mainly by drugs. Their management relies on the withdrawal of the culprit medication and supportive measures. Different pharmacotherapies have varied effects. However, data related to TEN and SJS in Saudi is limited. This study aimed to identify the causative agents, associated factors, and outcomes of TEN/SJS cases admitted to a teaching hospital (King Abdulaziz University) in Jeddah during the last 10 years.Methods: We retrospectively analyzed the data of TEN/SJS patients admitted to the hospital over the last 10 years.Results: We identified 12 patients with TEN/SJS. Of these, nine survived the condition and were discharged. The culprit medication was identified in eight of them, including antibiotics in six cases and Tegretol and allopurinol in one case each. Most of the patients received systemic steroids and intravenous immunoglobulins.Conclusion: TEN/SJS is mainly caused by medications of which antibiotics are the most implicated. Consistent with other studies, the mortality rate associated with TEN/SJS in Saudi is 25%. Limitations: restricted to a single center and small sample size.

Is It Stevens–Johnson Syndrome or MIS-C with Mucocutaneous Involvement?

Background. Severe acute respiratory syndrome coronavirus-2 (SARS-COV-2) can be present in the form of multisystem inflammatory disease in children. Case Presentation. A 25-month-old boy presented with fever, malaise, diffuse maculopapular rashes, and mucosal involvement during the COVID-19 pandemic. He was first diagnosed with Stevens–Johnson syndrome (SJS). Further evaluation revealed lymphopenia, thrombocytopenia, and elevated levels of C-reactive protein (CRP), ferritin, and fibrinogen. This was followed by a positive polymerase chain reaction (PCR) test for COVID-19. In addition to receiving initial care for SJS, he was treated for MIS-C, which led to his recovery after four days. Conclusion. COVID-19 infection should be considered in children with fever and dermatological features during the pandemic because it may cause different features of the multisystem inflammatory syndrome in children (MIS-C), suggestive of delayed hyperimmune response.

Multi-state model for predicting ocular progression in acute Stevens-Johnson syndrome/toxic epidermal necrolysis

This study aimed to clarify the etiologic factors predicting acute ocular progression in SJS/TEN, and identify patients who require immediate and intensive ophthalmological treatment. We previously conducted two Japanese Surveys of SJS/TEN (i.e., cases arising between 2005–2007 and between 2008–2010), and obtained the medical records, including detailed dermatological and ophthalmological findings, of 230 patients. Acute ocular severity was evaluated as none, mild, severe, and very severe. A multi-state model assuming the Markov process based on the Cox proportional hazards model was used to elucidate the specific factors affecting the acute ocular progression. Our findings revealed that of the total 230 patients, 23 (24%) of 97 cases that were mild at initial presentation worsened to severe/very severe. Acute ocular progression developed within 3 weeks from disease onset. Exposure to nonsteroidal anti-inflammatory drugs (NSAIDs) and younger patient age were found to be statistically significant for the progression of ocular severity from mild to severe/very severe [hazard ratio (HR) 3.83; 95% confidence interval (CI) 1.48 to 9.91] and none to severe/very severe [HR 0.98; 95% CI 0.97 to 0.99], respectively. The acute ocular severity score at worst-condition was found to be significantly correlated with ocular sequelae. Thus, our detailed findings on acute ocular progression revealed that in 24% of SJS/TEN cases with ocular involvement, ocular severity progresses even after initiating intensive treatment, and that in younger-age patients with a history of exposure to NSAIDs, very strict attention must be given to their ophthalmological appearances.

Toxic epidermal necrolysis associated with anticonvulsants

Background and Objetives: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are considered as a continuum of the same process. TEN or Lyell Syndrome is the most severe form. Both entities involve an acute mucocutaneous blistering reaction associated with systemic inflammation. Materials and Methods: We present a case of a young woman who developed TEN following concomitant treatment with valproate, lamotrigine, and phenobarbital. Despite the extensive mucocutaneous detachment (over 90%), prognostic evaluation was favorable (SCORTEN score 2; probability of survival 88%), and this patient evolved satisfactorily. Five days after admission, valproate was reinitiated without any subsequent adverse reaction. Results: Causality evaluation identified both lamotrigine and phenobarbital as “very probable” (ALDEN score = 6) causes and valproate as “very unlikely” (ALDEN score = 0) cause of TEN. Conclusions: SJS and TEN are true life-threatening medical emergencies. This case emphasizes the importance of early diagnosis and treatment, including the discontinuation of the causative agent, which can be lifesaving.

Matrix Metalloproteinase-9 and Topical Cyclosporine Are Associated with Conjunctival Microbiota Culture Positivity in Korean Patients with Stevens-Johnson Syndrome

BackgroundStevens-Johnson syndrome (SJS) is an abnormal immune-response causing extensive exfoliation of the mucocutaneous tissue including conjunctiva. While several factors are associated with the alteration of conjunctival microbiota, the conjunctiva of SJS patients are found to harbor a different microbiota compared to healthy subjects. We investigated the conjunctival microbiota of Korean SJS patients, and identified factors associated with the conjunctival microbiota and its positive culture.MethodsMedical records were retrospectively reviewed in 30 SJS patients who had undergone conjunctival swab culture sampling. Chronic ocular surface complications score (COCS), tear break-up time (TBUT), tear matrix metalloproteinase 9 (MMP9), and results of conjunctival swab culture were assessed.Results Positive culture was seen in 58.1%. Gram positive bacteria was most commonly isolated, among which Coagulase-negative Staphylococci (45.5%) and Corynebacterium species (40.9%) were predominantly observed. Tear MMP9 positivity was observed significantly more in the positive culture group (100%) compared to the negative culture group (75%) (P = 0.040). In patients who had repetitive cultures, positive- persistence group had more patients using topical cyclosporine compared to the negative-transition group (90.0% vs 33.3%, respectively, P = 0.041). No significant difference was found between COCS and conjunctival swab culture result, and the same as in TBUT and conjunctival swab culture result.ConclusionOur study suggest that tear MMP9 positivity may reflect the presence of an abnormal ocular surface microbiota and topical cyclosporine may be related to persistent culture positivity in SJS patients.

A Case Report of Steven Johnson Syndrome

Introduction: In 1922, two doctors, Albert Mason Stevens and Frank Johnson, examined purulent conjunctivitis.” Background: Stevens-Johnson syndrome was named after them as a result of their study. The incidence rate is 7 cases per million populations per year. Case Presentation: Master Yash Ghudam was brought to AVBRH by his parents with chief complaints of fever since 5 days and erythematous lesions all over body since 3 days. History of present illness: Patient was apparently alright 5 days back, and then he started having fever which was of high grade and was not associated with chills and rigor. Patient was treated on OPD basis and the symptoms of an unexplained disease in two young boys, aged 7 and 8, who had "an unusual, generalised eruption of continued fever, inflamed buccal mucosa, and extreme some antibiotic was given, but there was no relief, after 2 days there was ulcers formation inside the mouth for which some ointment and syrup becosule was started. But lesions were increasing. 3 days back the lesions first appeared on chest then got spread to legs and hands. For which patient was admitted in Chandrapur hospital from were the patient was referred to AVBRH for further management. Interventions: The patient was treated the patient was started on intravenous and orally Cortecosteroids, Omnacortil 10mg, Antibiotics- Inj. Ceftriaxone1gm IV 12 hourly [100mg/kg/day], inj. Amikacin 150mg IV 12 hourly [15mg/kg/day], Syp. Mucaine gel 2tsp BD – swish and swallow), Syp. Cital 2.5ml TDS, Tab. Chymoral Forte TDS, Inj. Pantop 20mg IV 24 hourly (1mg/kg/dose). Pandya’s Formula: Syp. Gelusil 5ml, Syp. Benadryl 5ml, Syp. Omnacortil 5ml. Skin allograft: It has been planned. Conclusion: In this study, we mainly focus on medical management and outstanding nursing care helped prevent farther complication. Overall, the patient's reaction was positive, though recovery time from Steven johnson syndrome varies from person to person, taking weeks, months, or even years. However, only a small number of people completely recover, while some have long-term consequences. She took a long time to get back on her feet.