Atypical Stevens-Johnson Syndrome in a Patient With Mycoplasma pneumoniae Infection: A Case Report.

Stevens-Johnson syndrome (SJS) is a serious condition involving the skin and mucous membranes and is characterized by extensive necrosis and detachment of the epidermis. We present a case report of atypical SJS occurring as a complication of Mycoplasma pneumoniae infection in a young adult patient. This case report aims to add to the limited body of literature that exists on the topic and remind clinicians of the possible diagnosis of atypical SJS in the setting of mucosal rash associated with M. pneumoniae infection.

Corneal descemetocele following Nd:YAG laser capsulotomy in a patient with Steven Johnson syndrome: A case report.

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

Steven-Johnson Syndrome/Toxic Epidermal Necrolysis is Associated with PD-1/PD-L1 Inhibitors Usage: A Case Series.

Aims/Background The increasing adoption of inhibitors of programmed cell death-1 (PD-1) and its ligand, programmed death-ligand 1 (PD-L1), in the treatment of multiple cancer types in China has started to garner broader attention due to the occurrence of immune-related adverse events (irAEs), especially life-threatening skin reactions such as Steven-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN). Isolated case reports have described SJS/TEN associated with PD-1/PD-L1 inhibitors usage. In this paper, we presented a series of cases of SJS/TEN following the use of PD-1/PD-L1 inhibitors in a dermatology hospital located in Zhejiang Province of China in the past several years, summarizing characteristics of these cases and providing a reference of management. Methods We retrospectively reviewed all the medical records of inpatients diagnosed with SJS/TEN in the Hangzhou Third People's Hospital from 2012 to 2024. We analyzed and compared the situation of SJS/TEN onset, types of PD-1/PD-L1 inhibitors used, score of severity, laboratory findings, and essential therapies of the patients who had received PD-1/PD-L1. Results We identified 12 SJS/TEN patients who had been treated with PD1/PD-L1 inhibitors: sintilimab had been used in six patients; tislelizumab in two cases; toripalimab, keytruda and cadonilimab each in one case; and an unknown prescription in one case. The longest duration between the first PD-1/PD-L1 inhibitor dose and the SJS/TEN diagnosis recorded was nine months whereas the shortest was 11 days. Half of the selected patients received chemotherapy at the same time. More than two types of therapies were applied to the cases, except for two cases with mild SJS. Conclusion This study unveils a potential, under-recognized cause of SJS/TEN in the cancer patients after analyzing the cases of SJS/TEN in cancer patients with prior exposure to PD-1/PD-L1 inhibitors. This paper also provides clue about the prominent features of SJS/TEN aforesaid, offering insights on the effective management measures for optimizing clinical safety.

The clinical outcomes of minor salivary gland transplantation for severe dry eye disease secondary to chronic Stevens-Johnson syndrome.

PURPOSE: To study the outcomes of minor salivary gland transplantation for severe dry eye disease secondary to chronic Steven Johnson Syndrome. METHODS: It was an ambispective, interventional case series conducted at Rajendra Prasad Centre for Ophthalmic Sciences, Delhi, India from 2022 to 2023 evaluating the outcomes of minor salivary gland transplantation with anchorage of the minor salivary glands to superior rectus muscle in twenty cases of severe dry eye disease secondary to chronic Steven-Johnson Syndrome. The pre-operative clinical parameters were compared to those at post-operative 1 year follow-up. RESULTS: At 1 year follow-up, there was an improvement in mean Schirmer-1 value (p = 0.0004), hyperemia score (p = 0.0004), keratinization score (p = 0.04), corneal epithelial defect score (p = 0.0004), corneal opacification score (p = 0.001), corneal neovascularization score (p = 0.001), palisades of Vogt score (p = 0.007), corneal keratinization score (p = 0.04) and corneal conjunctivalization score (p = 0.08). CONCLUSION: The minor salivary gland transplantation is a viable management option for cases with severe dry eye disease secondary to chronic Steven Johnson Syndrome with clinical improvement in corneal and conjunctival parameters of the ocular surface.

Ibuprofen-Induced Pancytopenia and Erythema Multiforme in an Elderly Female Patient.

Erythema multiforme (EM) is a delayed, cell-mediated cutaneous disease with varying clinical manifestations. It is most commonly associated with infections but can also be associated with medications, vaccines, and autoimmune diseases. Non-steroidal anti-inflammatory Drugs (NSAIDs) are commonly used analgesics that have rare associations with EM and pancytopenia. These adverse reactions to NSAIDs can obscure definitive diagnosis due to their rarity. We present a case where an elderly female patient taking 600mg of ibuprofen up to four times a day for shoulder bursitis developed EM and pancytopenia. In this case, a 75-year-old female with a medical history of atrial fibrillation, essential hypertension, non-insulin-dependent type 2 diabetes mellitus, and ischemic stroke with residual right-sided visual impairment presented to our Emergency Department in 2023 with neck swelling, skin rash, and ulceration of the oral cavity. She reported a generalized, targetoid body rash that occurred 15 days after she started taking ibuprofen regularly for left shoulder bursitis. No other medications were started before, after, or during this time period. CBC on admission was remarkable for a white blood cell count of 1.5x109/L, hemoglobin of 6.5 g/dL, and platelet count <10x109/L, consistent with pancytopenia. Ibuprofen was discontinued, and the patient was treated supportively with analgesia and packed red blood cell transfusions. Testing for HIV, antinuclear antibodies (ANA) panel, Hepatitis panel, and copper and zinc levels were negative. A biopsy of a targetoid lesion on the skin showed changes consistent with EM. Esophagogastroduodenoscopy revealed no actively bleeding lesions or ulcers in the stomach mucosa. The patient's blood counts eventually recovered with supportive treatment, and symptomatology improved. The patient was discharged six days after admission. Healthcare professionals should be aware of rare hematologic and immunologic side effects of NSAIDs, which may often be overlooked and misdiagnosed. More studies are needed to build on our wealth of knowledge regarding the etiology and management of EM, Steven Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN).

Outcome measurements in epidermal necrolysis: a systematic review.

Steven Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN), grouped together under the terminology of epidermal necrolysis (EN), are a spectrum of life-threatening dermatologic conditions. A lack of standardization and validation for existing endpoints has been identified as a key barrier to the comparison of these therapies and development of evidenced-based treatment. Following PRISMA guidelines, we conducted a systematic review of prospective studies involving systemic or topical treatments for EN, including dressing and ocular treatments. Outcomes were separated into mortality assessment, cutaneous outcomes, non-cutaneous clinical outcomes, and mucosal outcomes. The COSMIN Risk of Bias tool was used to assess the quality of studies on reliability and measurement error of outcome measurement instruments. Outcomes across studies assessing treatment in the acute phase of EN were varied. Most data came from prospective case reports and cohort studies representing the lack of available randomized clinical trial data available in EN. Our search did not reveal any EN-specific validated measures or scoring tools used to assess disease progression and outcomes. Less than half of included studies were considered "adequate" for COSMIN risk of bias in reliability and measurement error of outcome measurement instruments. With little consensus about management and treatment of EN, consistency and validation of measured outcomes is of the upmost importance for future studies to compare outcomes across treatments and identify the most effective means of combating the disease with the highest mortality managed by dermatologists.

Trastuzumab-induced toxic epidermal necrolysis in a Her-2-positive metastatic breast cancer patient-A rare case report.

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

High risk and low prevalence diseases: Stevens Johnson syndrome and toxic epidermal necrolysis.

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

Capecitabine and oxaliplatin induced Steven-Johnson syndrome following nivolumab in a patient of metastatic esophageal carcinoma.

Stevens-Johnson syndrome presents as mucocutaneous blistering and sloughing, which may follow a devastating clinical course. Although Stevens-Johnson syndrome has been reported following the administration of anticancer drugs, only a few cases induced by cytotoxic anticancer drugs, administered after immune checkpoint inhibitors, have been reported. The present report describes a case of Stevens-Johnson syndrome caused by capecitabine and oxaliplatin (CAPEOX) combination chemotherapy, in a patient with esophageal squamous cell carcinoma, who had been previously treated with nivolumab.

Etanercept treatment for pediatric toxic epidermal necrolysis induced by deflazacort: a case report and literature review.

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

Integrating antimicrobial photodynamic therapy into the adjuvant phototherapeutic approach for orofacial manifestations of toxic epidermal necrolysis and Stevens-Johnson syndrome.

Characterized by an immune reaction to medications, toxic epidermal necrolysis (TEN) and Steven-Johnson Syndrome (SJS) are potentially fatal mucocutaneous reactions, and their management remains challenging. Considering the promising studies regarding the use of laser in managing orofacial lesions, this study aimed to report two cases in which children presenting with TEN and SJS were treated using a combination of antimicrobial photodynamic therapy (aPDT) and photobiomodulation therapy (PBMT) concurrently with conventional supportive care. The treatment proposed herein resulted in significant clinical improvement of the children's oral condition within a few days, enabling the reintroduction of oral feeding. Within the limitations of the study, the cases reported suggest that the adjuvant combination of PBMT and aPDT may be beneficial for improving the oral condition of children afflicted with oral injuries induced by TEN and SJS.

Toxic Epidermal Necrolysis Following the Ingestion of Paracetamol in a Pediatric Patient: A Case Report.

Paracetamol is considered to be a relatively safe drug, even in the pediatric age group, at the recommended doses. Here we present a case of a six-year-old male presenting with symptoms and signs of Steven Johnson syndrome/toxic epidermal necrosis (SJS/TEN) following the ingestion of paracetamol. Steven Johnson syndrome/toxic epidermal necrosis is a potentially life-threatening dermatological emergency requiring intensive treatment. The patient was initially misdiagnosed as a case of chickenpox and was administered paracetamol. However, upon attending a tertiary care facility, he was diagnosed with TEN and treated with immunosuppressants. He recovered fully without any complications and was discharged within a week.

Phenytoin-Induced Toxic Epidermal Necrolysis.

Toxic epidermal necrolysis (TEN) is a rare fatal mucocutaneous blistering disorder that can have varied underlying triggers. The percentage of body surface area (BSA) that is impacted by erosive blistering is what separates it from Steven Johnson syndrome (SJS), both of which have the same underlying pathogenesis and are thought to exist on a continuum of disease with TEN being the more serious of the two. Medications are the most frequent cause of TEN/SJS and typically cause disease in both adults and children within eight weeks; however, the median exposure window is four days to four weeks. Nonsteroidal anti-inflammatory drugs, allopurinol, anticonvulsants including lamotrigine, phenytoin, levetiracetam and carbamazepine, antimicrobial sulfonamides, and the antiviral nevirapine are examples of medications that frequently cause TEN/SJS. Here, we are reporting a case of phenytoin-induced TEN highlighting the patient's excellent response to immunomodulating treatment despite 100% involvement of the BSA.

Stevens-Johnson Syndrome in Adult Patient Secondary to COVID-19 Infection: Case Report.

COVID-19 is a global pandemic caused by a novel zoonotic RNA virus named SARS-CoV-2. Various cutaneous manifestations associated with COVID-19 have been described, including urticarial rash, confluent erythematous rash, papulovesicular exanthem, chilblain-like acral pattern, livedo reticularis, and purpuric vasculitis pattern. Here, we are presenting a case of a 45-year-old male with mucocutaneous features of Stevens-Johnson syndrome.

Steven-Johnson Syndrome Induced by Lamotrigine and Valproic Acid in a Pediatric Patient: A Case Report.

Steven-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are part of a spectrum of severe cutaneous adverse reactions, secondary to infections or drug-induced. Although the use of antiseizure medications (ASMs) is a risk factor for the development of SJS/TEN, primary care physicians are not familiar with these cases in some countries. We report a case of SJS associated with ASMs in a nine-year-old girl with a history of difficult-to-control epilepsy, who required adjustment and change in medications.

Case report: Successful immunomodulators combined with electromagnetic field therapy in a patient with methazolamide-induced Steven Johnson syndrome/toxic epidermal necrolysis overlap.

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

Linezolid and Ciprofloxacin-Induced SJS/TEN (Stevens-Johnson Syndrome/Toxic Epidermolysis Necrosis) Overlap in a Patient With Borderline Personality Disorder During a Single Hospital Stay: A Difficult Case to Manage.

SJS/TEN (Stevens-Johnson syndrome/toxic epidermolysis necrosis) is a T-cell mediated hypersensitivity syndrome in which cytotoxic CD8+ cells react against keratinocytes, resulting in widespread apoptosis and cell necrosis. About 90% of these cases are attributed to drug reactions, while 10% are idiopathic. The disease is classified according to body surface area (BSA) involvement and the thickness of epidermal loss. We report a case of a female with borderline personality disorder on antipsychotic medication, who developed SJS/TEN overlap after taking ciprofloxacin for her urinary tract infection (UTI). Her condition improved with meticulous management, but after switching her antibiotic from intravenous clarithromycin to oral linezolid, she developed SJS/TEN again, this time with more severe involvement. She received active management involving a multidisciplinary approach. Her condition improved slowly and, after one month, her lesions began to heal, and she was discharged with advice not to use both antimicrobial drugs in the future.

An Unusual Case of Stevens-Johnson/Toxic Epidermal Necrolysis Overlap Syndrome in HER2 (Human Epidermal Growth Factor Receptor 2)-Positive Breast Cancer Patient Treated With Docetaxel.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but life-threatening drug-induced hypersensitivity reactions existing as a disease continuum based on the area of skin detachment. Following three cycles of treatment with docetaxel, a 60-year-old female with early-stage human epidermal growth factor receptor 2 (HER2)-positive breast cancer presented to the hospital with a flu-like illness and black crusting of the bilateral orbits, navel, and perianal region. Nikolsky sign was positive, and the patient was subsequently transferred to a specialized burn center for treatment of SJS/TEN overlap syndrome. There are a small number of cases documenting SJS/TEN following docetaxel administration in cancer patients.