Toxic epidermal necrolysis and Stevens-Johnson syndrome: A review*

Gerull, Roland; Nelle, Mathias; Schaible, Thomas

doi:10.1097/ccm.0b013e31821201ed

Cited by 157 publications

(153 citation statements)

References 159 publications

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“…[4][5][6] Although the pathogenesis is incompletely understood, SJS and its more severe form, toxic epidermal necrolysis (TEN), are the result of an inflammatory response that results in keratinocyte necrosis and perivascular lymphocyte infiltration. 7 SJS was classically ascribed to a medication hypersensitivity reaction; however, infectious etiologies, including Mycoplasma pneumoniae (Mp), are increasingly recognized as inciting agents. [8][9][10] SJS has an estimated incidence of 1 to 7 cases per million person-years, [11][12][13][14][15] although pediatric rates are not well described because of a lack of published information.…”

Section: Stevens-johnson Syndrome (Sjs) Ismentioning

confidence: 99%

Outbreak of Mycoplasma pneumoniae–Associated Stevens-Johnson Syndrome

et al. 2015

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BACKGROUND: Stevens-Johnson syndrome (SJS) is an uncommon, sporadic disease and outbreaks are rare. In November 2013, an outbreak of SJS was identified at Children's Hospital Colorado.METHODS: Outbreak cases were children aged 5-21 with a discharge diagnosis of SJS admitted from September 1 to November 30, 2013. Medical charts were reviewed using standardized data collection forms. Respiratory specimens were tested for viruses and Mycoplasma pneumoniae (Mp) by polymerase chain reaction (PCR). We conducted a separate 4-year retrospective case-control study comparing hospitalized SJS cases with and without evidence of Mp infection.RESULTS: During the outbreak, 8 children met SJS criteria. Median age was 11.5 years (range 8-16 years); 5 (63%) were boys and 5 (63%) were Mp-PCR-positive. Of the 5 PCR-positive children, none had preceding medication exposure, and all had radiographic pneumonia. All outbreak Mp isolates were macrolide susceptible. The retrospective case-control analysis showed that Mp-associated SJS episodes (n = 17) were more likely to have pneumonia (odds ratio [OR] 10.0, confidence interval [CI] 1.3-5.1), preceding respiratory symptoms (OR 30.0, CI 1.6-72.6), an erythrocyte sedimentation rate $35 mg/dL (OR 22.8, CI 2.1-244.9), and #3 affected skin sites (OR 4.5, CI 1.2-17.4) than non-Mp-associated SJS episodes (n = 23). CONCLUSIONS:We report the largest outbreak of SJS in children, which was also predominately associated with Mp infection. Mp-associated SJS was associated with a distinct clinical presentation that included less extensive skin disease, an elevated erythrocyte sedimentation rate, and evidence of a preceding respiratory infection.WHAT'S KNOWN ON THIS SUBJECT: StevensJohnson syndrome (SJS) is a rare and severe immunologic phenomenon characterized by rash and mucous membrane disease. SJS may be triggered by medications and, less commonly, by infections such as Mycoplasma pneumoniae (Mp). Outbreaks of SJS are exceedingly rare. WHAT THIS STUDY ADDS:We describe the largest SJS outbreak reported in children, which was also Mp-associated. In the first case-control study of this disease, we identify predictors of Mp-associated SJS versus non-Mp-associated SJS, including fewer skin lesions, pneumonia, and elevated erythrocyte sedimentation rate.

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Section: Stevens-johnson Syndrome (Sjs) Ismentioning

confidence: 99%

Outbreak of Mycoplasma pneumoniae–Associated Stevens-Johnson Syndrome

et al. 2015

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“…Patients with skin lesions affecting between 10 and 30% of their body surface area are considered to have SJS/TEN overlap (1). These epidermal detachments are considered life-threatening (2) and are most frequently manifested by adverse drug reaction. In addition to occurring as a reaction to drugs, these skin eruptions are also associated with underlying infectious diseases, and include the cutaneous manifestations of disseminated candidiasis (3,4), Mycoplasma pneumoniae (5), Chlamydia pneumoniae (6), cytomegalovirus infection (7) and human immunodeficiency virus (8,9).…”

Section: Introductionmentioning

confidence: 99%

Clinical characteristics and treatment outcome of Stevens-Johnson syndrome and toxic epidermal necrolysis

Chantaphakul

Sanon

Klaewsongkram

2015

Experimental and Therapeutic Medicine

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Abstract. Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are erythematous skin lesions with blister formation accompanied by mucosal involvement. These conditions are considered to be life-threatening illnesses. Understanding the clinical presentation, risk factors, treatment options and results will be advantageous for physicians in the management of patients in the future. The aim of the present study was to review and analyze the clinical manifestations, drug implications, treatment and outcome of patients with SJS and/or TEN who had been hospitalized in a tertiary care center. All hospitalized patients with SJS and/ or TEN during a 5-year period were retrospectively reviewed. The clinical severity was graded according to the score of toxic epidermal necrolysis (SCORTEN) scale. Clinical symptoms, diagnosis, possible precipitating factors, management and outcome data were collected for analysis. A total of 43 patients (mean age, 49.5 years) were hospitalized and classified into the SJS group (55.8%), SJS/TEN overlap group (20.9%) and TEN group (23.3%). The majority of the patients (90.7%) had mucocutaneous eruptions associated with oral drug administration. Allopurinol, anticonvulsants and antibiotics were the most common causative agents for the mucocutaneous eruption. Twenty-eight patients (65.1%) were treated with corticosteroids. The mortality rate was 6.9%. Comparison between the survival group and the non-survival group revealed that patient age >70 years (P=0.014) and body surface area involvement >20% (P<0.01) were the significant factors associated with mortality. The use of systemic steroids was higher in the survival group in comparison with the non-survival group (65.1 vs. 0%, respectively; P=0.014). The mucocutaneous eruptions in SJS and TEN are mostly caused by medication. With early recognition and treatment, the mortality rate in this study was lower than that in previous reports. Patient age and the area of mucocutaneous involvement were significant factors associated with mortality.

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“…The disease runs an unpredictable course, an initially benign-appearing dermatosis can progress rapidly. 10,11 The present study found that dermatological system was most commonly affected followed by gastrointestinal (GI) and vascular system. More than 90% of SJS occurs within the first 2 months of AED use.…”

Section: Discussionmentioning

confidence: 88%

“…They range from minor maculopapular exanthem (MPE) to severe life-threatening reactions like Drug reaction eosinophilia and systemic symptoms (DRESS), Stevens-Johnson Syndrome (SJS) and Toxic epidermal Necrolysis (TEN), and manifest within a few hours to weeks of the AED being initiated. 10,11 Hence this study was undertaken to evaluate the pattern of ADRs due to AEDs.…”

mentioning

confidence: 99%

Pattern of adverse drug reaction to antiepileptic drugs in a tertiary care hospital

Akalu

Belavadi

2017

Int J Basic Clin Pharmacol

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Background: Adverse drug reactions (ADRs) are a major cause of morbidity and mortality, and are the leading cause of hospital admission. The overall rate of ADRs is estimated to be 6.5% and 28% of these ADRs are preventable. Antiepileptic drugs (AEDs) are authorized for several therapeutic indications and are highly prescribed. ADRs due to AEDs range from minor maculopapular exanthem (MPE) to severe life-threatening reactions like Drug reaction eosinophilia and systemic symptoms (DRESS) and Stevens-Johnson syndrome (SJS). Objective of the study was to evaluate the pattern of ADRs reported with AEDs in an adverse drug reaction monitoring centre (AMC) of a tertiary care hospital.Methods: Retrospective analysis of the records was done for a period 48 months from January 2013 to December 2016. During this period, all the ADRs caused by AEDs reported to the AMC were included in the study. The study evaluated the pattern of ADRs due to AEDs. The study also assessed the gender-wise distribution, predilection for various systems, causality, severity, and preventability of ADRs. Data was analysed using descriptive statistics.Results: A total of 319 ADRs were reported by spontaneous reporting during the entire study period. Out of the total 319 ADR reports received, antiepileptic drugs related ADRs were 35 (11%). Antiepileptic drugs which caused the ADRs included phenytoin, carbamazepine, clobazam and lorazepam. The most common system affected was dermatological (60%), followed by gastrointestinal system (17.14%), vascular system (11.42%), blood (5.8%), respiratory system (5.8%) and central nervous system (2.9%). Among the dermatological ADRs, SJS accounted for 11 cases of which 10 cases were due to phenytoin and one case was due to carbamazepine. DRESS syndrome due to phenytoin was documented in one case.Conclusions: AEDs are the most commonly prescribed drugs for various indications. Uses of AEDs are accompanied by ADRs which vary from mild rashes and itching to SJS and DRESS/TEN. Post-marketing surveillance of the AEDs is important for compliance, therapeutic efficacy and ultimately safety of the patient.

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Toxic epidermal necrolysis and Stevens-Johnson syndrome: A review*

Abstract: Profound knowledge of exfoliative skin diseases is needed to improve therapy and outcome of these life-threatening illnesses.

Cited by 157 publications

References 159 publications

Outbreak of Mycoplasma pneumoniae–Associated Stevens-Johnson Syndrome

Outbreak of Mycoplasma pneumoniae–Associated Stevens-Johnson Syndrome

Clinical characteristics and treatment outcome of Stevens-Johnson syndrome and toxic epidermal necrolysis

Pattern of adverse drug reaction to antiepileptic drugs in a tertiary care hospital

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