Your browser doesn't support javascript.
loading
Show: 20 | 50 | 100
Results 1 - 20 de 259
Filter
1.
Arch Dermatol Res ; 316(8): 564, 2024 Aug 23.
Article in English | MEDLINE | ID: mdl-39177823

ABSTRACT

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe mucocutaneous reactions, usually to drugs, characterized by blistering and epithelial sloughing. SCORTEN is an established prognosticator index employed in SJS/TEN patients to evaluate their severity degree and mortality risk. Many studies done in the recent past have indicated that neutrophil-lymphocyte ratio (NLR) is related to disease activity in several dermatological diseases. Hence, this study has been performed to correlate the NLR of each patient with their respective SCORTEN values and assess whether NLR can be used as a prognostic marker in SJS/TEN. A single centre, retrospective, 4 year study was conducted at a tertiary care hospital. The required clinical and laboratory data were obtained from existing IP records of all cases of SJS/TEN disorders admitted in the last 4 years in our hospital between May 1st 2019 and April 30th 2023. The correlation coefficient and p value were analysed using the Spearman's rank correlation. The total sample size of the study was 22 patients. A female preponderance (59.1%) with an age range between 10 to 74 years was noted. Drugs were the main triggering factor in all the patients and antiepileptics were the most commonly implicated drug group. On statistical analysis a weak positive correlation (r = 0.182) between NLR and SCORTEN was noted, however p value was insignificant (p = 0.417). Further, mean ± SD of NLR was found to be higher in group II (patients with SCORTEN ≥ 3) as compared to group I (patients with SCORTEN < 3). On correlating NLR with each group separately, p value still remained insignificant. Elevation in NLR value reflects the systemic inflammation, but its role in predicting the severity of the disease needs further research involving larger sample size.


Subject(s)
Lymphocytes , Neutrophils , Severity of Illness Index , Stevens-Johnson Syndrome , Humans , Neutrophils/immunology , Female , Male , Retrospective Studies , Middle Aged , Lymphocytes/immunology , Adult , Prognosis , Aged , Adolescent , Child , Stevens-Johnson Syndrome/blood , Stevens-Johnson Syndrome/diagnosis , Stevens-Johnson Syndrome/immunology , Young Adult , Biomarkers/blood , Lymphocyte Count
2.
Cureus ; 16(7): e63922, 2024 Jul.
Article in English | MEDLINE | ID: mdl-39104984

ABSTRACT

Drug reactions with eosinophilia and systemic symptoms (DRESS) syndrome and Stevens-Johnson syndrome-toxic epidermal necrolysis (SJS-TEN) are reactive entities of aberrant cytotoxic immunologic reactions to exogenous medications. While they are conventionally seen as distinct, separate conditions, we present a case of a rare evolution of DRESS syndrome into SJS-TEN in the setting of simultaneous amoxicillin-clavulanate initiation and long-term sildenafil use in a 66-year-old South Asian female with a known history of prior DRESS syndrome and pulmonary arterial hypertension. We discuss the conditions leading to her unique clinical presentation and provide considerations for future clinical encounters.

3.
Front Immunol ; 15: 1415830, 2024.
Article in English | MEDLINE | ID: mdl-39091503

ABSTRACT

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


Subject(s)
Anti-Bacterial Agents , Humans , Retrospective Studies , Male , Female , Anti-Bacterial Agents/adverse effects , Middle Aged , Adult , Aged , Stevens-Johnson Syndrome/etiology , Stevens-Johnson Syndrome/mortality , Stevens-Johnson Syndrome/drug therapy , Young Adult , China/epidemiology , Adolescent , Drug Hypersensitivity Syndrome/etiology
4.
Burns ; 2024 Jul 20.
Article in English | MEDLINE | ID: mdl-39127578

ABSTRACT

BACKGROUND: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) represent severe manifestations of a potentially life-threatening spectrum defined by a desquamating rash of the skin and mucous membranes. This study was prompted by the observed increase in the off-label use of lamotrigine as a causal agent in SJS/TEN in our regional burn center. METHODS: A retrospective cohort of 48 patients presenting to the Connecticut Burn Center from 2015-2022 with suspicion for SJS/TEN were reviewed for age, sex, causative drug, presenting symptoms, hospital course, biopsy confirmation, length of stay, comorbidities, and 30-day mortality. Descriptive statistical analysis was conducted to identify trends in causative agent, clinical presentation, and mortality. RESULTS: Thirty patients in our cohort received a final diagnosis of SJS/TEN. While antibiotics remain the most frequent cause of SJS/TEN across the study period (33.3 %, n = 10), the incidence of cases attributable to lamotrigine increased from 1 case between 2015 and 2018 (6.7 %) to 6 cases between 2019 and 2022 (40 %). In 2020 alone, 50 % of all cases were attributable to lamotrigine (n = 4). Of the patients where lamotrigine was implicated, 71.4 % (n = 5) were prescribed lamotrigine for off-label use in the treatment of non-bipolar mood disorders. The average lamotrigine-associated SJS/TEN patient was younger (p < 0.001), had fewer comorbidities, and was more likely to be female than the general SJS/TEN population. CONCLUSION: Off-label use of lamotrigine is emerging as a major driver of SJS/TEN with notable changes in patient demographics. Further research is necessary to understand how changing trends in the patient population will impact clinical course and optimal management.

5.
Cureus ; 16(6): e63123, 2024 Jun.
Article in English | MEDLINE | ID: mdl-39055403

ABSTRACT

Swyer-James syndrome (SJS), also termed MacLeod syndrome, is an acquired secondary unilateral hyperlucency of the lung due to childhood lung infections. This disorder can be diagnosed in children; however if there are few or no symptoms, diagnosis can be missed and can then be detected later in adult life as an incidental finding. We present here the case reports of two patients, where one of them had a unique presentation of unilateral hyperlucency on a chest radiograph and a bilateral mosaic pattern on CT lung but with no history of childhood infections and another case with unilateral hyperlucency of the lung with the history of childhood infection were diagnosed as SJS. This article is important as it highlights the significant radiological finding in accurately diagnosing this condition, when the presenting complaint and past history are inconclusive, thereby guiding proper management.

6.
Cureus ; 16(6): e63353, 2024 Jun.
Article in English | MEDLINE | ID: mdl-39077250

ABSTRACT

Stevens-Johnson Syndrome (SJS) constitutes a rather uncommon, and rarely fatal hypersensitivity reaction that primarily impacts the skin and mucous membranes and in certain cases may be attributed to drug administration. The aim of this article is to present a case of etoricoxib-induced SJS in a 46-year-old, female patient. The patient presented herself, as a medical emergency, to the Department of Oral Medicine/Pathology, School of Dentistry, Aristotle University of Thessaloniki, Greece, reporting pain, especially acute pain while eating certain foods, discomfort, dysphagia, and a wound in the left half of the hard palate. The clinical examination revealed a broad ulcer, in the left half of the hard palate as well as multiple ulcerations and erosions in the upper and lower lip. Her medical history was clear; however, the patient mentioned to have received etoricoxib, due to severe back pain, one day prior to our clinical examination. The patient received methylprednisolone 16 mg, twice per day, for two days, followed by methylprednisolone 8 mg, twice per day, for two more days. Her symptoms resigned and since the connection between etoricoxib and SJS was established, the patient was advised to avoid etoricoxib and be wary of adverse effects, when taking drugs especially non-steroidal anti-inflammatory medication. This is one of the first case reports in the literature, linking etoricoxib administration with the emergence of SJS, highlighting the importance of pharmacovigilance. The up-to-date registration of drug-induced adverse effects is of immense importance to protect future patients. SJS does not have a defined treatment strategy. Therefore, most patients are given supportive care and symptomatic treatment, which most commonly involves corticosteroids and antivirals such as acyclovir.

8.
Arch Dermatol Res ; 316(6): 233, 2024 May 25.
Article in English | MEDLINE | ID: mdl-38795205

ABSTRACT

Immune checkpoint inhibitor (ICI) therapies carry the risk of major immune-related adverse events (irAEs). Among the most severe irAEs is epidermal necrosis that may clinically mimic Stevens-Johnson syndrome (SJS) and toxic epidermal necrosis (TEN). The aim of this study was to provide a summary of the clinical and histological features of ICI-associated epidermal necrosis, with a special focus on factors associated with fatal outcomes in cases of extensive disease. A total of 98 cases, 2 new cases and 96 reported on PubMed and in the literature, of ICI-associated epidermal necrosis were assessed. Development of epidermal necrosis occurred between 1 day and 3 years after starting ICI therapy, with an average onset of 13.8 weeks for patients with limited (< 30% BSA) and 11.3 weeks for those with extensive (≥ 30% BSA) involvement, and a median onset of 5.8 weeks and 4 weeks respectively. A preceding rash was seen in 52 cases and was more common in extensive cases. Mucosal involvement was only reported in 65% of extensive cases but was significantly associated with fatal reactions. Co-administration of cytotoxic chemotherapy was associated with more extensive disease. Recovery was observed in 96% and 65% of those with limited and extensive involvement respectively and no specific therapy was associated with improved survival. Young age was significantly associated with poor outcomes in extensive disease, the average age of surviving patients was 64.5 years old versus 55.1 years old for deceased patients, p < 0.01. Both superficial perivascular and interface/lichenoid inflammatory infiltrates were commonly seen. These findings suggest that ICI-associated epidermal necrosis should be considered a distinct clinical entity from drug-induced SJS/TEN.


Subject(s)
Immune Checkpoint Inhibitors , Necrosis , Stevens-Johnson Syndrome , Humans , Immune Checkpoint Inhibitors/adverse effects , Stevens-Johnson Syndrome/pathology , Stevens-Johnson Syndrome/etiology , Stevens-Johnson Syndrome/immunology , Stevens-Johnson Syndrome/diagnosis , Necrosis/chemically induced , Epidermis/pathology , Epidermis/drug effects , Epidermis/immunology , Middle Aged , Female , Male , Aged , Adult
10.
Ocul Surf ; 34: 9-21, 2024 May 03.
Article in English | MEDLINE | ID: mdl-38703818

ABSTRACT

PURPOSE: Stevens-Johnson syndrome (SJS) is characterised as an immuno-inflammatory condition with potentially blinding ocular sequelae. Therefore, we have investigated the ocular surface immune cell profile and correlated it with secreted tear molecular factors and clinical ocular sequelae in SJS patients. METHODS: 21 patients (42 eyes) with chronic ocular SJS and 16 healthy controls (20 eyes) were included in the study. Severity, types of keratopathies and ocular surface (OS) manifestations were determined. OS wash samples from study subjects were used to determine the status of 13 immune cell subsets using flow cytometry. Levels of 42 secreted immuno-inflammatory factors were measured by flow cytometry-based multiplex ELISA in tear samples. RESULTS: Neutrophils (Total, activated), neutrophils/NK cells ratio, neutrophils/T cells ratio were significantly (p < 0.05) elevated in SJS, while, proportions of T cells and NKT cells were significantly lower in SJS patients. Positive association between neutrophils and chronic ocular surface complication score (COCS) was observed, whereas, a negative association was noted between NK cells and COCS. Tear fluid levels of IL-6, IL-8, IL-18, IFNα/ß/γ, TNFα, LIF, IL-8, HGF, sTNFR-I, NGAL, Granzyme, Perforins, MMP9/TIMP1 ratio were significantly higher in SJS. Loss of Limbal niche correlated significantly with immune profile and clinical sequelae. Increased neutrophils, decreased NK cells and specific set of altered secreted immuno-inflammatory mediators including bFGF, and IL-8 were observed in SJS patients with different types of keratopathies compared to those without keratopathy. CONCLUSION: Distinct ocular surface immune profile variations were observed to correlate with clinical stages of chronic ocular SJS. Our findings uncover novel mechanisms and potential for targeted therapy in chronic ocular SJS patients.

11.
BMC Ophthalmol ; 24(1): 205, 2024 May 06.
Article in English | MEDLINE | ID: mdl-38711013

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.


Subject(s)
Corneal Diseases , Stevens-Johnson Syndrome , Visual Acuity , Humans , Stevens-Johnson Syndrome/surgery , Stevens-Johnson Syndrome/physiopathology , Retrospective Studies , Female , Male , Adult , Visual Acuity/physiology , Middle Aged , Young Adult , Adolescent , Corneal Diseases/surgery , Corneal Diseases/physiopathology , Treatment Outcome , Child , Corneal Transplantation/methods , Follow-Up Studies , Keratoplasty, Penetrating/methods , Postoperative Complications , Limbus Corneae/surgery
12.
Allergy ; 79(8): 2128-2143, 2024 Aug.
Article in English | MEDLINE | ID: mdl-38682250

ABSTRACT

PURPOSE: This study aims to elucidate the tear proteome and understand the underlying molecular mechanisms involved in the ocular complications following Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN). METHODS: Mass spectrometry (MS) was performed to quantify the tear fluid proteins from chronic SJS/TEN patients (n = 22 eyes) and age- and gender-matched controls (n = 22 eyes). The candidate proteins were validated using ELISA (n = 80 eyes) in tear samples and immunohistochemistry (IHC; n = 12) in eyelid margin specimens. These proteins were compared for significant differences based on age, gender, disease duration, and ocular severity. RESULTS: A total of 1692 tear fluid proteins were identified, of which 470 were significantly differentially regulated in chronic SJS/TEN. The top 10 significantly upregulated proteins were neutrophil secretions including neutrophil elastase (p < .0001), defensin (p < .0001), and matrix metalloproteinase 8 (p < .0001). The presence of neutrophils was confirmed by the upregulation of IL-8 (p < .001) in tears, a key cytokine known for recruiting neutrophils. Additionally, positive expression of myeloperoxidase was observed in the keratinized eyelid margins of SJS/TEN to validate the presence of neutrophils. Among 41 unique proteins identified by MS, IL-36γ (p < .01) was expressed in three SJS/TEN patients and was confirmed in SJS/TEN tears and eyelid margins by ELISA and IHC, respectively. IL-36γ was specifically expressed in the superficial layers of eyelid margin keratinized conjunctiva. The majority of the significantly downregulated proteins were lacrimal gland secretions such as lacritin (p < .0001) and opiorphin (p < .002). Neutrophil elastase (p < .02) was significantly elevated in patients with severe eyelid margin keratinization. CONCLUSION: Our observations indicate a clear correlation between eyelid margin keratinization and the expression of IL-36γ, potentially mediated by neutrophils recruited via IL-8. Future experimental studies are needed to test the role of therapies targeting IL-8 and/or IL-36γ in reducing eyelid margin keratinization and its associated ocular complications in SJS/TEN.


Subject(s)
Interleukin-1 , Neutrophils , Stevens-Johnson Syndrome , Tears , Humans , Female , Male , Neutrophils/metabolism , Neutrophils/immunology , Interleukin-1/metabolism , Stevens-Johnson Syndrome/metabolism , Stevens-Johnson Syndrome/pathology , Adult , Tears/metabolism , Middle Aged , Chronic Disease , Inflammation/metabolism , Aged , Young Adult
13.
Cureus ; 16(3): e55318, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38562325

ABSTRACT

Cutaneous adverse drug reactions (CADRs) are one of the most broadly studied and rigorously researched conditions in recent dermatological advancements. Also termed as "toxidermia," they are heavily involved and are of utmost importance to be understood and studied in the modern healthcare industry. In simple terms, they are dermatological manifestations which result from systemic drug administration to patients. Since allopathy is influenced by the medicines and drugs provided to the patients, cutaneous skin eruptions are a common occurrence in recent times. It is a need of the hour to understand the causative factors for such skin eruptions and the correct management and handling of such disorders to provide better healthcare to patients. The withdrawal of the causative drug which induces the reaction plays a key role in treatment. The risk factors are to be thoroughly studied, and dosages must be in accordance with the patient's situation. They are some of the common public health problems. The age group which is affected is highly variable as people from all age groups can be affected. Those who are affected comprise approximately 10% of all hospitalized patients, and it is also observed in about 1-4% of people who are on multiple medications.

14.
Cureus ; 16(3): e56868, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38659518

ABSTRACT

Pneumocystis pneumonia (PCP) primarily affects immunosuppressed patients, with trimethoprim-sulfamethoxazole (TMP-SMX) commonly used for prophylaxis. However, there is insufficient information on PCP occurrence despite TMP-SMX prophylaxis. We encountered a 57-year-old woman with locally advanced breast cancer developing PCP despite prophylactic intake of TMP-SMX, during treatment with prednisolone for Stevens-Johnson syndrome (SJS) induced by pembrolizumab. This case underscores the need to pay attention to the possibility of PCP development even during TMP-SMX prophylaxis. Dosage and duration adjustments according to the patient's condition and weight may be required.

15.
Cureus ; 16(3): e56735, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38646265

ABSTRACT

The management of oral mucosal pain in Stevens-Johnson syndrome (SJS), known for its severe mucocutaneous reactions, is a significant challenge due to the paucity of effective treatments reported in the literature. This case report aims to help fill this gap by describing the effective use of continuous intravenous fentanyl for the relief of severe oral mucosal pain in a patient with SJS. A patient with postoperative recurrence of cervical cancer developed SJS following chemotherapy. She had severe oral mucosal pain that was not relieved by 12.5 mcg/hour fentanyl transdermal patch, a regular medication. This pain was rated 10/10 on the Numerical Pain Rating Scale (NRS), and the patient had dysphagia and difficulty speaking. On admission, intravenous methylprednisolone (1000 mg/day), oral lip treatment with dexamethasone ointment, and oral rinses with azulene-lidocaine mixture were started. Analgesic treatment consisted of a 12.5 mcg/hour fentanyl transdermal patch of the regular medication and 1000 mg/dose of intravenous acetaminophen twice daily. Due to the inadequate efficacy of the transdermal patch, fentanyl was switched from the transdermal patch to a continuous intravenous fentanyl infusion at 20 mcg/hour on day three of admission. This adjustment significantly reduced pain intensity, which decreased to NRS 5/10 on day six of admission, and the patient was able to drink water and speak. Pain relief preceded clinical improvement of stomatitis. Grade 1 somnolence occurred after the start of intravenous fentanyl, but improved with follow-up. There were no other adverse effects such as respiratory depression. This case highlights the potential of intravenous fentanyl in the treatment of oral mucosal pain associated with SJS, although further studies are needed to confirm these findings and to develop comprehensive pain management protocols.

16.
Am J Emerg Med ; 81: 16-22, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38631147

ABSTRACT

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.


Subject(s)
Emergency Service, Hospital , Stevens-Johnson Syndrome , Stevens-Johnson Syndrome/diagnosis , Stevens-Johnson Syndrome/therapy , Stevens-Johnson Syndrome/etiology , Humans , Prevalence
17.
Ocul Surf ; 32: 173-181, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38490474

ABSTRACT

PURPOSE: To clinically define a subset of patients with chronic ocular Stevens-Johnson syndrome non-responders (SJS-NR) and analyze their cytokine profile compared to clinical responders (SJS-CR). METHODS: A total of 32 SJS cases (n = 32, 64 eyes) managed over a period of three years were segregated into clinical responders (n = 24, 48 eyes) and non-responders (n = 8, 16 eyes). Cases were determined as non-responders based on persistent, refractory, and non-mechanical inflammation of the conjunctiva. Age- and sex-matched healthy controls (n = 25, 50 eyes) were recruited. Tear specimens collected using Schirmer's strip were profiled for 27 cytokines using an immunoassay-based 27-bioplex array. RESULTS: Tear cytokine profiling revealed 18 cytokines to be differentially expressed in SJS-NR compared to SJS-CR. While PDGF-BB, IL-4, IL-1ß, VEGF, IL-12p70, IFN-γ, IL-9, and IL-1RA were upregulated, GM-CSF, eotaxin, IP-10, IL-10, MCP-1, G-CSF, IL-6, IL-13, and bFGF were downregulated in SJS-NR compared to SJS-CR. The cytokines IL-13, IL-10, and IP-10 were decreased in both SJS-NR and SJS-CR compared to controls. CONCLUSION: The inflammation in SJS-NR continues to worsen despite the correction of mechanical causes, resulting in progressive deterioration of the cornea. The cytokine profile of SJS-NR was remarkably different from that of SJS-CR, indicating a T helper 2-type protective proliferative response and an impaired migratory potential of the conjunctival epithelium. These factors could possibly lead to poor healing of the corneal epithelium in a markedly pro-inflammatory and pro-angiogenic milieu. The top four differentially expressed cytokines, PDGF-BB, IL-4, IL-10, and IL-6, are proposed as potential biomarkers of SJS-NR.


Subject(s)
Cytokines , Stevens-Johnson Syndrome , Stevens-Johnson Syndrome/drug therapy , Stevens-Johnson Syndrome/pathology , Cytokines/analysis , Cytokines/metabolism , Humans , Male , Female , Young Adult , Adult , Middle Aged , Tears/chemistry , Cornea/metabolism , Cornea/pathology , Down-Regulation , Immunomodulation , Immunosuppression Therapy , Mycophenolic Acid/therapeutic use
18.
Front Immunol ; 15: 1289492, 2024.
Article in English | MEDLINE | ID: mdl-38510251

ABSTRACT

Sjögren's syndrome (SjS) is a systemic, highly diverse, and chronic autoimmune disease with a significant global prevalence. It is a complex condition that requires careful management and monitoring. Recent research indicates that epigenetic mechanisms contribute to the pathophysiology of SjS by modulating gene expression and genome stability. DNA methylation, a form of epigenetic modification, is the fundamental mechanism that modifies the expression of various genes by modifying the transcriptional availability of regulatory regions within the genome. In general, adding a methyl group to DNA is linked with the inhibition of genes because it changes the chromatin structure. DNA methylation changes the fate of multiple immune cells, such as it leads to the transition of naïve lymphocytes to effector lymphocytes. A lack of central epigenetic enzymes frequently results in abnormal immune activation. Alterations in epigenetic modifications within immune cells or salivary gland epithelial cells are frequently detected during the pathogenesis of SjS, representing a robust association with autoimmune responses. The analysis of genome methylation is a beneficial tool for establishing connections between epigenetic changes within different cell types and their association with SjS. In various studies related to SjS, most differentially methylated regions are in the human leukocyte antigen (HLA) locus. Notably, the demethylation of various sites in the genome is often observed in SjS patients. The most strongly linked differentially methylated regions in SjS patients are found within genes regulated by type I interferon. This demethylation process is partly related to B-cell infiltration and disease progression. In addition, DNA demethylation of the runt-related transcription factor (RUNX1) gene, lymphotoxin-α (LTA), and myxovirus resistance protein A (MxA) is associated with SjS. It may assist the early diagnosis of SjS by serving as a potential biomarker. Therefore, this review offers a detailed insight into the function of DNA methylation in SjS and helps researchers to identify potential biomarkers in diagnosis, prognosis, and therapeutic targets.


Subject(s)
Autoimmune Diseases , Sjogren's Syndrome , Humans , DNA Methylation , Epigenesis, Genetic , Gene Expression Regulation
20.
Cureus ; 16(1): e52222, 2024 Jan.
Article in English | MEDLINE | ID: mdl-38347997

ABSTRACT

Toxic epidermal necrolysis (TEN) is a rare and life-threatening cutaneous disease, frequently triggered by drugs. Allopurinol is one of the most frequent drugs associated with TEN, which implies detachment of a significant amount of the body surface area (BSA) and has a high morbidity and mortality associated with it. We present the case of a 68-year-old female with a recent diagnosis of hyperuricemia who started treatment with allopurinol. A week later, she presented to the emergency department with an extensive maculopapular exanthema with blisters and skin detachment. After the exclusion of other etiologies, the diagnosis of allopurinol-induced TEN was made, with 35% of BSA involvement. Due to the severity of the clinical condition, she was admitted to intensive care and treated with corticoids that had no response. So, she was started on immunoglobulins and transferred to a burn unit. She developed sepsis with multiorgan failure and required supportive treatment. She was discharged after a month, and physical rehabilitation was needed. This clinical case highlights the severity of allopurinol hypersensitivity that may happen and the importance of an accurate diagnosis and treatment for this rare disease.

SELECTION OF CITATIONS
SEARCH DETAIL