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Chinese Medical Journal May 2017Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening diseases with high mortality rates. This study was designed to analyze the...
BACKGROUND
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening diseases with high mortality rates. This study was designed to analyze the pathogenic factors, clinical manifestations, complications, treatment, and prognosis of SJS/TEN and to explore the differences between surviving and deceased patients.
METHODS
SJS/TEN patients admitted to Beijing Friendship Hospital from January 2006 to December 2015 were included in the study. Patients' data were retrospectively analyzed. Comparative studies were performed on the survival group and the deceased group, and Fisher's exact probability test was used for statistical analysis.
RESULTS
Among the 88 patients included, 40 (45.5%) were male with a mean age of 45 ± 18 years. Forty-eight (54.5%) had SJS, 34 (38.6%) had SJS/TEN, and 6 (6.8%) had TEN. Fifty-three (60.2%) cases were caused by medications, mainly antibiotics (n = 24) followed by traditional Chinese medicines (n = 7). Forty-two cases (47.7%) developed visceral damage. Eighty-two patients improved or recovered and were discharged from hospital, and six patients died. Comparative studies on the survival group and the deceased group showed that the presence of malignant tumor ( χ2 = 27.969,P < 0.001), connective tissue diseases ( χ2 = 9.187, P= 0.002), previous abnormal liver/kidney functions ( χ2 = 6.006, P= 0.014), heart rate >100 times/min ( χ2 = 6.347, P= 0.012), detached skin area >20% ( χ2 = 5.594, P= 0.018), concurrent mucosal involvement at the mouth, eyes, and external genitals ( χ2 = 4.945, P= 0.026), subsequent accompanying liver/kidney damage ( χ2 = 11.839, P= 0.001, and χ2 = 36.302,P < 0.001, respectively), and SCORTEN score >2 ( χ2 = 37.148,P < 0.001) increased the risk of death.
CONCLUSIONS
SJS/TEN is mainly caused by medications, and nearly half of patients develop visceral damage. Multiple factors increase the mortality risk.
Topics: Adult; Anti-Bacterial Agents; Connective Tissue Diseases; Eye; Female; Genitalia; Humans; Kidney; Liver; Male; Middle Aged; Mouth; Retrospective Studies; Skin; Stevens-Johnson Syndrome
PubMed: 28469101
DOI: 10.4103/0366-6999.204929 -
Korean Journal of Ophthalmology : KJO Jun 2021To study the ocular manifestations, its severity and sequelae in patients with Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN).
PURPOSE
To study the ocular manifestations, its severity and sequelae in patients with Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN).
METHODS
Prospective study of 44 consecutive patients (30 SJS and 14 TEN) presenting in the acute phase of the disease. Patients were evaluated by dermatologist as well as physician for systemic status, skin lesions and mucosal involvement. Detailed history taking, visual acuity, ophthalmic evaluation (lid margin, corneal, conjunctival changes, tear film and ocular surface). Ocular severity score (OSS) was assessed at baseline (acute) and at 6 months (chronic / OSS6), graded as mild, moderate and severe.
RESULTS
Mean age was 28.15 ± 15.78 years. Sixty-five eyes of 33 patients were included for final analysis. Thirty-eight patients (86.4%) had ocular manifestations. Drugs were the most common causative factor (95.4%). At base line mild, moderate, and severe OSS was seen in 43.1%, 44.6%, and 12.3% eyes. At 6 months mild, moderate, and severe OSS was seen in 44.6%, 7.7%, and 6.2% of eyes. There was a significant correlation between age of the patient and OSS at 6 months (p = 0.02). Younger age had higher chronic OSS. Patients with TEN had higher acute (p = 0.001) and chronic (p = 0.001) OSS than SJS. Three mucosal surface involvement associated with higher acute and chronic OSS (p = 0.001). No long-term ocular complications observed in 27 / 65 (41.5%) eyes. Acute OSS correlated significantly with chronic OSS, at 1 and 6 months (p = 0.001).
CONCLUSIONS
Greater severity of the disease, more number of mucosal surfaces involved and shorter symptom lag correlated with more severe acute and chronic ocular manifestations. The severity of lid margin involvement and corneal involvement in acute stage were good predictors of severity of chronic ocular findings. Initial severity of ocular involvement correlated with severity of ocular sequelae.
Topics: Adolescent; Adult; Child; Conjunctiva; Disease Progression; Humans; Infant; Prospective Studies; Retrospective Studies; Stevens-Johnson Syndrome; Visual Acuity; Young Adult
PubMed: 33596622
DOI: 10.3341/kjo.2020.0118 -
Annals of the Academy of Medicine,... Dec 2021
Topics: Anticonvulsants; Humans; Lamotrigine; Singapore; Stevens-Johnson Syndrome
PubMed: 34985104
DOI: 10.47102/annals-acadmedsg.2021326 -
American Journal of Ophthalmology Sep 2016To report the occurrence of corneal ectasia (ECT) in patients with history of Stevens-Johnson syndrome (SJS), and to make the case for an association between these 2...
PURPOSE
To report the occurrence of corneal ectasia (ECT) in patients with history of Stevens-Johnson syndrome (SJS), and to make the case for an association between these 2 diagnoses. We also report the impact of prosthetic replacement of the ocular surface ecosystem (PROSE) treatment on visual acuity (VA) in these patients.
DESIGN
Retrospective cohort study.
METHODS
A manufacturing database of PROSE patients from 2002 to 2014 at Boston Foundation for Sight (BFS), a single-center clinical practice, was reviewed to identify patients with diagnoses of both SJS and ECT.
RESULTS
Nine patients were identified with diagnoses of both SJS and ECT. In each case, review of the medical record revealed that diagnosis of SJS preceded that of ECT. The prevalence of ECT in this population exceeded that in the general population (P < .0001). Videokeratography was available for 13 eyes in 7 patients; using Krumeich's classification of keratoconus, 3 eyes were found to be at stage 1, 3 at stage 2, 1 at stage 3, and 6 at stage 4. Sixteen of 18 eyes underwent PROSE treatment. Of these 16 eyes, initial median VA was 20/200 (range, count fingers to 20/20; logMAR 1.0). Median VA after PROSE customization was 20/30 (range, 20/60-20/15; logMAR 0.1761, P < .0025).
CONCLUSIONS
ECT occurs at a higher-than-expected rate in patients with a history of SJS. PROSE treatment improves VA in these patients. The basis of the association between SJS and ECT is considered, as well as the role of plausible contributory factors such as corneal microtrauma and matrix metalloproteinases.
Topics: Adult; Cohort Studies; Contact Lenses; Corneal Diseases; Corneal Topography; Dilatation, Pathologic; Female; Humans; Male; Prostheses and Implants; Retrospective Studies; Stevens-Johnson Syndrome; Visual Acuity; Young Adult
PubMed: 27386788
DOI: 10.1016/j.ajo.2016.06.039 -
Frontiers in Immunology 2022Acute graft-versus-host disease (aGVHD) is a severe and fatal complication after orthotopic liver transplantation (OLT). Clinical manifestations of severe aGVHD can...
BACKGROUND
Acute graft-versus-host disease (aGVHD) is a severe and fatal complication after orthotopic liver transplantation (OLT). Clinical manifestations of severe aGVHD can resemble drug-induced Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN), and there are also various medications, such as antibiotics and immunosuppressants, used after transplantation, causing a diagnostic dilemma. Furthermore, there have been no standardized diagnostic and therapeutic strategies for OLT-aGVHD due to its rarity.
CASE SUMMARY
A 52-year-old man presented with generalized maculopapular eruptions, fever, and pancytopenia 1 month after OLT and 4 days after taking sulfamethoxazole/trimethoprim. After assessment of the scoring criteria for drug causality of drug allergy, histopathological findings of skin biopsy, lymphocyte activation test of the potential offending drug, and microchimerism study, the diagnosis was in favor of aGVHD mimicking SJS/TEN. Considering severe sepsis, the anti-tumor necrosis factor alpha (TNF-α) agent, etanercept, was used to replace tacrolimus and corticosteroid. Skin lesions resolved gradually after anti-TNF-α biologics rescue; tacrolimus and corticosteroid therapy were re-administrated after controlling sepsis. Pancytopenia recovered and the patient was discharged in a stable condition.
CONCLUSION
We demonstrated a diagnostic strategy for OLT-aGVHD. Targeting therapy with anti-TNF-α blockade and a temporary withdrawal of traditional immunosuppressants may be among effective and safe therapeutic options of OLT-aGVHD for those with severe sepsis.
Topics: Adrenal Cortex Hormones; Graft vs Host Disease; Humans; Immunosuppressive Agents; Liver Transplantation; Male; Middle Aged; Pancytopenia; Sepsis; Stevens-Johnson Syndrome; Tacrolimus; Tumor Necrosis Factor Inhibitors; Tumor Necrosis Factor-alpha
PubMed: 36059444
DOI: 10.3389/fimmu.2022.917782 -
JAMA Dermatology Jan 2021This case series documents the incidence of various nail changes as sequalae to Stevens-Johnson syndrome/toxic epidermal necrolysis.
This case series documents the incidence of various nail changes as sequalae to Stevens-Johnson syndrome/toxic epidermal necrolysis.
Topics: Adult; Aged; Chronic Disease; Disease Progression; Female; Follow-Up Studies; Humans; Incidence; Male; Middle Aged; Nail Diseases; Nails; Stevens-Johnson Syndrome; Survivors
PubMed: 33295942
DOI: 10.1001/jamadermatol.2020.4664 -
Molecular Vision 2022This study sought to investigate the association of molecular markers with chronic ocular sequelae in Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN).
PURPOSE
This study sought to investigate the association of molecular markers with chronic ocular sequelae in Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN).
METHODS
One hundred SJS/TEN patients (200 eyes) with confirmed diagnosis were enrolled between July 2011 and July 2015 from a tertiary eye-care hospital, and their clinical histories were noted. Each eye was scored for severity of manifestation on a scale of 0-5. Peripheral blood samples were collected for DNA followed by screening for interleukin (IL-4, IL-13, IL-4R) polymorphisms, HLA-A locus allele typing, and sera to detect levels of the apoptotic markers granulysin and sFas L.
RESULTS
Of the 100 enrolled patients (53 males/47 females; age range: 6-58 years), the incriminating drugs were non-steroidal anti-inflammatory (52%), antibiotics (10%), sulphonamides (8%), anti-epileptics (6%), and unknown (24%). Significant differences in the frequencies of IL-4R polymorphism, HLA-A*3301, HLA-A*02, and HLA-A*2402 alleles, and elevated levels of granulysin and sFas L were observed in patients compared to controls. The ocular complications of conjunctival keratinization (p=0.004) showed an association with IL-13 promoter region (IL-13a) genotypes.
CONCLUSIONS
The study highlights the possible association of interleukin-13 with severity-graded chronic sequelae and the role of HLA-A alleles- HLA-A*3301, HLA-A*02, and HLA-A*2402 in SJS/TEN causation and manifestation. Screening of these alleles may help caregivers to identify markers associated with severe and lifelong ocular complications, and help in appropriate treatment and management of the condition.
Topics: Male; Female; Humans; Child; Adolescent; Young Adult; Adult; Middle Aged; Stevens-Johnson Syndrome; Interleukin-13; Eye; Genetic Association Studies; HLA-A Antigens; Genetic Predisposition to Disease
PubMed: 37089698
DOI: No ID Found -
Allergology International : Official... Dec 2010Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening adverse reactions, which could be induced by a variety of drugs. It was proposed... (Review)
Review
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening adverse reactions, which could be induced by a variety of drugs. It was proposed that human leukocyte antigen (HLA)-restricted presentation of antigens (drugs or their metabolites) to T lymphocytes initiates the immune reactions of SJS/TEN. However, the genetic susceptibility and the exact pathogenesis were not clear until the recent studies. We first identified that HLA-B*1502 is strongly associated with carbamazepine (CBZ)-induced SJS/TEN and HLA-B*5801 with allopurinol-SJS/TEN in Han Chinese. The same associations had been validated across different human populations. For the downstream danger signals, Fas-Fas ligand (FasL) and perforin/granzyme B had been advocated as cytotoxic mediators for keratinocyte death in SJS/TEN. However, expression levels of these cytotoxic proteins from the skin lesions were too low to explain the distinct and extensive epidermal necrosis. Our recent study identified that the granulysin, a cytotoxic protein released from cytotoxic T cells or natural killer (NK) cells, is a key mediator for disseminated keratinocyte death in SJS/TEN. This article aims to provide an overview of both of the genomic and immunologic perspectives of SJS/TEN. These studies give us a better understanding of the immune mechanisms, biomarkers for disease prevention and early diagnosis, as well as providing the therapeutic targets for the treatments of SJS/TEN.
Topics: Allopurinol; Apoptosis; Biomarkers; Carbamazepine; Cytotoxicity, Immunologic; Genetic Predisposition to Disease; Granzymes; HLA-B Antigens; HLA-B15 Antigen; Humans; Keratinocytes; Killer Cells, Natural; Polymorphism, Genetic; Stevens-Johnson Syndrome; T-Lymphocytes, Cytotoxic
PubMed: 20962567
DOI: 10.2332/allergolint.10-RAI-0261 -
Gaceta Medica de Mexico 2012The Stevens-Johnson syndrome (SJS) and the toxic epidermal necrolysis (TEN) are clinical conditions manifesting as adverse cutaneous reaction to drugs in majority of... (Review)
Review
The Stevens-Johnson syndrome (SJS) and the toxic epidermal necrolysis (TEN) are clinical conditions manifesting as adverse cutaneous reaction to drugs in majority of cases, constituting the same clinical spectrum, differing only in the severity of epidermolysis; both conditions are distinguished by their severity and extensiveness of skin lesions; it can also involve mucous membranes of eyes, respiratory, digestive and urogenital tracts. Two per 1,000,000 are affected annually, among them approximately 20% are children and both of them are considered as potentially fatal medical emergency conditions. Even though the condition was described 89 years ago, until now the exact pathophysiology has not been completely explained. An immune-mediated mechanism has been implicated in its origin, which involves cytotoxic lymphocytes, cytokines, Fas-ligand in keratinocyte apoptosis; genetic makers also has been identified in some racial groups (HLA-B*1520, HLA-B*5801) in relationship with specific susceptibility to certain drugs such as carbamazepine, allopurinol. In children there are no uniform criteria for classification of the skin lesions, neither for the treatment, however recently the authors describe better response of the patients with use intravenous immunoglobulin (IGIV).
Topics: Adolescent; Child; Child, Preschool; Humans; Infant; Practice Guidelines as Topic; Stevens-Johnson Syndrome
PubMed: 22820360
DOI: No ID Found -
Allergology International : Official... Mar 2006Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN, Lyell's syndrome) are now considered to be distinct clinical entities within a spectrum of adverse... (Review)
Review
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN, Lyell's syndrome) are now considered to be distinct clinical entities within a spectrum of adverse cutaneous drug reactions of increasing severity based on their surface of skin detachment. Within this spectrum, SJS which can be considered as a minor form of TEN is characterized by less than 10% body surface area of skin detachment, and an average reported mortality of 1-5%, whereas TEN is characterized by more than 30% skin detachment, and an average reported mortality 25-35%. Both SJS and TEN are characterized morphologically by the rapid onset of keratinocyte cell death by apoptosis, a process that results in the separation of the epidermis from the dermis. Recent evidence is supportive of a role for inflammatory cytokines and the death receptor Fas and its ligand FasL in the pathogenesis of keratinocyte apoptosis during TEN. This Fas-mediated keratinocyte apoptosis that is the last step culminating in epidermal detachment in TEN can be inhibited in vitro by antagonistic monoclonal antibodies to Fas, and by intravenous immunoglobulins (IVIG) which have been shown to contain natural anti-Fas antibodies. Consequently, over the last few years, numerous case reports and 9 non-controlled clinical studies containing 10 or more patients have analyzed the therapeutic effect of IVIG in TEN. Taken together, although each study has its potential biases, 7 of 9 such studies point towards a benefit of IVIG used at doses greater than 2 g/kg on the mortality associated with TEN. These studies should serve as the basis for designing an appropriate prospective trial or for conducting a metaanalysis in the near future, in order to determine the therapeutic efficacy of IVIG in TEN.
Topics: Drug Eruptions; Fas Ligand Protein; Humans; Immunoglobulins, Intravenous; Keratinocytes; Stevens-Johnson Syndrome
PubMed: 17075281
DOI: 10.2332/allergolint.55.9