Stevens-Johnson syndrome

Soluble FAS Ligand: A Discriminating Feature between Drug-Induced Skin Eruptions and Viral Exanthemas

Skin eruptions have been reported with the use of all antiepileptic drugs and there is a significant risk of cross-reactivity between these agents in causing serious eruptions such as Stevens-Johnson's syndrome. Gabepentin is usually considered a safe agent for patients with a previous history of drug allergies and there have been no cases of skin eruption reported to the gabapentin post marketing surveillance. We report a patient who had severe Stevens-Johnson's syndrome induced by phenytoin and later by carbamazepine. Subsequent use of gabapentin also resulted in a skin eruption which was limited to the lower extremities but without systemic or mucosal involvement. This case suggests that patients with a strong history of drug-induced idiosyncratic reactions may experience such reactions to gabapentin as well.

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Background. Stevens — Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but severe immune-mediated adverse skin reactions, most commonly caused by drug products or infections. Objective. To study the risk factors of SJS and TEN development in patients who were on inpatient treatment in the department of allergology of the City Clinical Hospital № 21 of the Republic of Bashkortostan in 2014—2023. Material and methods. A retrospective analysis of the case histories of 229 patients, considering the affected area of the body’s surface, was carried out. Patients were divided into 3 groups: 83 had SJS, 41 — SJS/TEN combination, 105 — TEN. The following parameters were analyzed with regard to the risk of developing SJS and TEN: age and sex, past medical history and concomitant diseases prior application of drug products, maximum detachment of the epidermis as a percentage of the body’s affected area. Results. Cardiovascular diseases (CVDs) (14.5%), gastrointestinal diseases (8.4%) and diabetes mellitus (6%) were risk factors for SJS development; CVDs (24.4%), peripheral vascular disease (14.6%) and diabetes mellitus (12.2%) — risk factors for SJS/TEN development; diabetes mellitus (16.2%), CVDs (12.4%), HIV (9.5%) and malignant tumors (9.5%) — risk factors for developing TEN. Drug products were the cause of SJS and SJS/TEN development (48.2 and 48.8%, respectively). Pharmaceuticals (63.8%) and viral infection (22.9%) were mainly the etiological factor of TEN development. Conclusion. The presence of HIV, malignant neoplasms and diabetes mellitus are more significant for patients with TEN. Peripheral vascular disease is more common in patients with SJS/TEN than with TEN. Drug products and viral infection were etiologically more significant for TEN than for SJS and SJS/TEN; in contrast, the cause of disease could not be identified more often in patients with SJS and SJS/TEN.

Background: Stevens-Johnson syndrome (SJS) has been reported as a serious adverse effect in patients treated with vancomycin or linezolid, and there is currently a lack of real-world studies comparing specific differences in adverse effects of SJS. Methods: According to the FDA’s Adverse Event Reporting System (FAERS), from January 2004 to July 2021, the data of suspected SJS after the use of vancomycin and linezolid were analyzed by imbalance and Bayesian analysis. The onset time, fatality rate and hospitalization rate of vancomycin-associated SJS and linezolid-associated SJS were also investigated. Results: 276 cases of vancomycin-related SJS reports and 63 cases of linezolid-related SJS reports were identified. These two drugs are more common in middle-aged patients (45–64 years) than other age groups, and less common in underage children (<18). Among them, linezolid-related SJS is more common in middle-aged and elderly patients (45–74 years old) than other groups. Except for unspecified data, in vancomycin-associated SJS cases, there are more men than women (49.28% vs 43.84%), while in linezolid-associated SJS cases, the proportion of men and women is almost equal (44.44%). From the point of view of the areas where adverse reactions were reported, about 1/2 of the reports on Vancomycin-related SJS came from North America, and 1/3 of the reports came from Europe. The median onset time of Linezolid-related SJS was 5 days (interquartile range [IQR] 2–7.75), which was significantly earlier than that of Vancomycin-related SJS (12 days, IQR 4–20) (Mann-Whitney test, p < 0.0001). There were no significant differences in mortality and hospitalization rates after vancomycin and linezolid caused SJS. Conclusion: The analysis of faers data provides a comprehensive overview of the adverse reactions of SJS caused by the use of vancomycin and linezolid, and can warn clinical workers to timely intervene and continuously monitor the patients at risk of SJS when using such drugs.

Stevens - Johnson syndrome (SJS) is a severe, episodic, acute mucocutaneous reaction that is most often elicited by drugs and occasionally by infections. We report a case of 29 years old woman who developed Stevens - Johnson syndrome following the ingestion of Nimesulide tablets. Nimesulide is a nonsteroidal anti-inflammatory (NSAID) drug with antipyretic and analgesic properties. To the best of our knowledge there have been no previous reports of SJS induced by the ingestion of Nimesulide in the medical literature. This case highlights the importance of Nimesulide and other NSAIDS as the possible cause of Stevens - Johnson syndrome which needs to be borne in mind by the treating physician in cases of drug reactions. Key words Nimesulide; Stevens - Johnson syndrome. DOI: http://dx.doi.org/10.3126/njdvl.v9i1.5765 NJDVL 2010; 9(1): 25-27

Stevens-Johnson syndrome and toxic epidermal necrolysis (TEN) are part of a spectrum of mucocutaneous disorders with similar clinical features. The incidence of TEN and StevensJohnson syndrome has been estimated at 0.4 to 1.2 and 1 to 6 cases per million person-years, respectively.[1-3] Factors such as race, age, gender and socioeconomic status do not appear to affect the epidemiology of the two disorders. The pathophysiology of these skin syndromes remains unknown. Stevens-Johnson syndrome is considered to be a milder form of TEN. StevensJohnson syndrome typically presents as small blisters on dusky pruritic macules on the face and upper trunk, occurring 1 to 3 weeks after drug exposure. The rash progresses rapidly to include the epithelium of the gastrointestinal and respiratory tracts and eyes, and it usually reaches its maximum within hours to days.[4] Detachment of less than 10% of the epidermis is considered StevensJohnson syndrome, while involvement of greater than 30% of the body surface is diagnosed as TEN. Fever is usually higher in TEN than in StevensJohnson syndrome. Mortality depends upon the extent of skin necrosis and damage to other systems and is usually below 5% for Stevens-Johnson syndrome and as high as 30% in cases of TEN.[1,5] Patients may be severely dehydrated with electrolyte abnormalities, and anaemia, lymphopenia and pre-renal azotemia are common. Bacterial colonisation of the skin and decreased immune response increases the likelihood of sepsis, the major cause of death.[4] Thirty-five percent of patients with TEN and fewer with Stevens-Johnson syndrome experience ocular sequelae including formation of synechiae between the eyelids and the conjunctiva, corneal erosions and scarring, and punctate keratitis.[6] Neutropenia, visceral involvement and high blood urea nitrogen levels are associated with a poorer prognosis. While both are primarily associated with drug use, infections, including mycoplasma pneumonia and viruses, are also implicated in the Stevens-Johnson syndrome. The drugs most often causing Stevens-Johnson syndrome and TEN include sulfonamides, aminopenicillins, phenytoin and nonsteroidal anti-inflammatory drugs (NSAIDs). Despite its wide clinical use for cough suppression and pain control, codeine has not been implicated as a cause of Stevens-Johnson syndrome. We report a paediatric case of Stevens-Johnson syndrome associated with codeine phosphate.

Fever, Rash, and Systemic Symptoms: Understanding the Role of Virus and HLA in Severe Cutaneous Drug Allergy

Background: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe eruptive skin reactions that can cause death. The incidence of SJS and TEN cases in the United States is 1.5–9.6 per 1,000,000 per year. Drugs are the primary etiology of SJS and TEN. Some drugs are at high risk and used frequently. The SJS and TEN mortality rates were relatively high, with SJS 4.8%, SJS / TEN overlap 19.8%, and TEN 14.8%. In Indonesia, there are lack of studies on the SJS and TEN. This study is needed to determine the epidemiological profile of SJS and TEN. Purpose: This study aimed to describe SJS and TEN patients' profiles. Methods: Drug-induced SJS and TEN cases from January 2016 to December 2019 were evaluated from the medical records patients' profile, incidence, suspected drugs, risk factors, and comorbidities of SJS and TEN were described. Result: There were 28 SJS and TEN patients, comprising of 24 SJS patients (85.7%), 3 TEN patients (10.7%), and 1 SJS overlapping TEN patients (3.5%). The most common suspected drugs were paracetamol (22.2%), carbamazepine (20.4%), cefadroxil (8.8%), and ciprofloxacin (8.8%). Women (53.5%) experienced more severe drug eruptions than men (46.4%). The largest age group was 25–44 years (35.7%). Most comorbidities were epilepsy (21%), diabetes (15.7%), hypertension (15.7%), and stroke (15.7%). Conclusion: The most common manifestation was SJS with paracetamol as the most common suspected drug, followed by carbamazepine.

Background:Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe cutaneous adverse reactions (SCARs). There is scant literature on the characteristics and causes of these conditions among the Nigerian population. Here, we describe the epidemiology, associated morbidity and mortality, and culpable drugs in SJS and TEN cases using the National Pharmacovigilance (NPC) database in Nigeria.Methods:A retrospective review of the NPC database was done to analyze SJS and TEN cases reported over a period of 14 years. Annual reports, age and sex of patients, type of reporter, suspects and concomitant drugs, time to onset (TTO) of the reactions, and outcome of SJS and TEN were evaluated.Results:The NPC received a total of 24,015 adverse drug reaction (ADR) reports. SJS and TEN accounted for 284 (0.1%) of the total reports, of which 254 (89.4%) were SJS and the remainder were TEN. Females (n = 184, 64.8%) and individuals aged 19–40 years (n = 181, 63.7%) were the most affected by SJS and TEN. Antiretrovirals, followed by antibiotics, were the most common drug classes reported to cause SJS and TEN, with nevirapine (n = 174, 40.7%) and co-trimoxazole (n = 143, 33.5%) being the most widely implicated drugs. Among patients with reported outcomes, 73 (28.7%) SJS and 3 (10.0%) TEN cases recovered without sequelae, at the time of reporting. Severity of the SCAR was reported for only 171 (69.0%) cases, of which 12 (4.7%) and 8 (26.7%) resulted in death (Grade 5) among SJS and TEN cases, respectively.Conclusions:Antiretroviral and antibiotics were the commonly reported offending group of drugs for SJS and TEN cases. Nevirapine and co-trimoxazole were the commonly reported suspect drugs. SJS and TEN were reported most frequently in females and in patients aged 19–40 years, indicating that drug surveillance and counseling in these groups of patients may be beneficial.

The incidence of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) has been reported to be between 0.95 and 1 per 1000 individuals with AIDS. Accessibility to a cohort of individuals with HIV with known drug exposure (including drug, dose, and time of exposure) and collection of adverse-event information may provide an opportunity to determine an incidence rate of SJS and TEN. The primary objective of this analysis was to determine the incidence of confirmed SJS and TEN in a cohort of Canadian HIV patients who were receiving HIV and HIV-related medications. This was a retrospective analysis of an HIV cohort. The Ontario HIV Treatment Network (OHTN) cohort population was eligible for this analysis. A search of the OHTN database was conducted to determine whether cases with a diagnosis of SJS or TEN were included. Search terms included 'TEN,' 'SJS,' 'epidermal necrolysis,' and 'erythema multiforme.' All SJS and TEN cases recorded in the OHTN database between January 1995 and August 2008 were obtained. Diagnostic criteria for SJS and TEN were established and two reviewers examined the medical records to confirm the SJS or TEN diagnosis. Drug exposure and utilization were documented. Incidence rates for the entire cohort were calculated. Seventeen cases over seven OHTN study sites were identified from an approximate cohort sample size of 3700. There were 15 men (88%). The mean ± SD age was 51.6 ± 11.3 years and time since HIV diagnosis was 16.1 ± 4.4 years. Only one patient reported experiencing a previous SJS or TEN episode. Of the 17 cases, clinical experts diagnosed five cases as true SJS and/or TEN, two cases were labeled as indeterminant, and the remaining cases were considered not SJS or TEN. Among the confirmed cases, drugs taken included nevirapine, trimethoprim/sulfamethoxazole (cotrimoxazole), stavudine (d4T), and clarithromycin. The incidence of SJS and/or TEN was 5-7 per 3710 or approximately 1-2 per 1000 individuals in this cohort with HIV. Careful diagnosis of this adverse event is required for an accurate measure of incidence and to avoid false inflation of the incidence.

Stevens - Johnson syndrome (SJS) has manifestation through the exfoliation of epidermis and mucosal tissue. Ocular surface is usually affected in acute and chronic stage. The patients are usually suffered from chronic ocular sequelae including symblepharon, limbal stem cell deficiency, etc. Furthermore, ocular microbiome may also be altered in SJS. This is prospective, age and sex matched analytical study which including 20 chronic SJS patients and 20 healthy subjects for specimen collection from inferior conjunctiva for microbiome analysis by conventional cultures and Next-Generation Sequencing (NGS) methods. Significant higher proportion of positive-cultured specimen was demonstrated in SJS group (SJS group 60%, healthy 10%, p-value = 0.001). In addition, NGS which providing high-throughput sequencing has demonstrated the greater diversity of microbial species. The higher proportion of pathogenic microorganisms including Pseudomonas spp., Staphylococcus spp., Streptococcus spp., Acinetobacter spp. was shown in SJS group. Ocular surface in SJS is usually occupied by more diverse microorganisms with increased proportion of pathogenic species. This condition may affect chronic inflammation and opportunistic infections in SJS group. In order to prevent and treat infection in these patients, appropriate antibiotics based on bacterial examination should be considered as the first-line treatment in the SJS patients.

Stevens Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but unpredictable severe hypersensitive reactions with high significant mortality and potentially life-threatening. Indonesia has a large population but study of SJS/TEN in Indonesia is still limited. The purpose of our study was to describe data the patients profile, incidence, mortality, and related diseases based on ICD-10 nomenclature system of all patients with SJS and TEN, retrospectively. Medical records of hospitalized patients with SJS (L51.1), TEN (L51.2) and SJS/TEN overlap (L51.3) from January 1, 2012 to December 31, 2017 in dr.Saiful Anwar General Hospital Malang, Indonesia were reviewed. The study has obtained 75 SJS/TEN (63 SJS; 12 TEN) patients data from 2012 – 2017 with incidence rate was 12.5 cases per years. No SJS/TEN overlap was observed. This study demonstrates that SJS/TEN were more frequent in middle adulthood than other age groups. In SJS, female were more frequent than male. In contrast, male more susceptible to TEN than female. The mortality rate was 9.5% in SJS and 16.7% in TEN or 10.67% in total. Based on ICD-10 classification, eye and adnexa disorder, infection, and endocrine, nutritional, metabolic abnormality were the most frequent complication in SJS and TEN.

To report a rare case of drug induced overlap of Stevens-Johnson syndrome and Toxic Epidermal Necrosis Syndrome exacerbated by cephalexin. In this case report, we present a 65-year-old female who had come to the hospital with complaints of Sloughing of the skin and redness all over the body with raised body temperature. She was on therapeutic Phenytoin to prevent the post-surgical complications of Communicating Hydrocephalus. After a detailed examination, it was found that the patient had misemployed with an overdose of Phenytoin. The patient was found with nikolsky sign and diagnosed as Stevens- Johnson syndrome and Toxic Epidermal Necrosis overlap. This case report emphasizes phenytoin induced Stevens-Johnson syndrome and Toxic Epidermal Necrosis syndrome exacerbated by cephalexin. By witnessing this phenomenon, we could figure out the association between cephalexin and Stevens-Johnson syndrome- Toxic Epidermal Necrosis syndrome overlap. The Immediate dismissal of the offending agent and commencement of supportive care was found to be effective.

Stevens-Johnson syndrome (SJS) and its more advanced form, Toxic Epidermal Necrolysis (TEN) are severe adverse cutaneous reactions that predominantly involve skin and mucous membranes. In view of the current dearth of documented knowledge, this study the first of its kind from India was designed to categorically distinguish and compare the different associated factors and clinical manifestations relevant to the development of SJS/TEN syndrome among the immunocompromised (Human Immunodeficiency Virus 1 seropositive) patients in Eastern India. 16 out of 29 patients (55.1%) were found to be suffering from drug induced SJS or TEN, while the rest (44.8%) had severe pathogenic involvements. Neviraprine use was found to be the major cause among drug involved SJS (53.8%) and TEN (66.66%) cases followed by allopurinol use. The frequency of incidence of kidney disease (67% in SJS and 54% in TEN) and neurological impairment (42% SJS and 37%TEN) was found to be significantly higher among the SJS patients whereas that of hepatitis (38% SJS and 47% TEN) , ocular dysfunction (49% SJS and 63% TEN) and pulmonary dysfunction (47% SJS and 53% TEN) were higher among the TEN patients. This study also had another prominent motive, which is to elucidate the possible role of human cytomegalovirus in triggering the exfoliative inflammatory disease conditions among these patients. The incidence rate of SGPT and SGOT were significantly higher in TEN patients than in SJS (p=0.001 and p=0.002) . Mean sodium level in blood was within normal range in both the groups whereas potassium and chloride levels were much higher than normal Out of the 29 HIV seropositive patients with SJS/TEN we studied, only 4 (13.7%) had an active HCMV infection. In detailed study of the associated laboratory and clinical parameters, cytokine analysis profile, Immuno-histologic findings and rigorous analysis of the investigation reports identified HCMV infection as the probable trigger behind SJS/TEN development in these patients.