Pdf on dec 1, 20, haur yueh lee and others published epidemiology of stevensjohnson syndrome and toxic epidermal necrolysis in southeast asia find, read and cite all the research you need. Toxic epidermal necrolysis ten and stevens johnson syndrome sjs are severe adverse cutaneous drug reactions that predominantly involve the skin and mucous membranes. May 20, 2016 toxic epidermal necrolysis ten is a rare, acute, and lifethreatening mucocutaneous disease that is usually drug related. Epidemiology of erythema exsudativum multiforme majus, stevensjohnson syndrome, and toxic epidermal necrolysis in germany 19901992.
Ikzf1, a new susceptibility gene for cold medicinerelated stevensjohnson syndrometoxic epidermal necrolysis with severe mucosal involvement. Stevensjohnson syndrome sjs and toxic epidermal necrolysis ten are acute, lifethreatening necrotic skin reactions caused by. A phenytoin induced toxic epidermal necrolysis ten a case. No large scale epidemiological data are available for this disorder in india. Stevensjohnson syndrome and toxic epidermal necrolysis can be recurrent for different drugs. Copyright 20 by the asian pacific journal of tropical disease. Publications from 1995 to 2011 describing sjs and ten in indian. This multicenter retrospective study included patients with sjsten seen by inpatient consultative dermatologists at 18 academic medical centers in. Toxic epidermal necrolysis ten is a rare skin condition that causes you to lose your outer layer of skin. Aug 22, 2014 toxic epidermal necrolysis ten is an acuteonset, potentially lifethreatening, idiosyncratic mucocutaneous reaction, usually occurring after commencement of a new medication. People with sjs or ten caused by a medication have a better prognosis the earlier the causative medication is withdrawn. Stevensjohnson syndrome and toxic epidermal necrolysis usually begin 1 to 3 weeks after the start of a drug if caused by a drug with fever, headache, cough, keratoconjunctivitis inflammation of the conjunctiva and the cornea in the eyes, and body aches.
Stevensjohnson syndrome sjs and toxic epidermal necrolysis ten are rare severe cutaneous drug reactions. Stevensjohnson syndrome sjs is a rare, immunemediated, skin reaction that results in blistering of skin and extensive epidermal detachment. Seasonal variation of stevensjohnson syndrome and toxic epidermal necrolysis associated with trimethoprimsulfamethoxazole. Pdf epidemiology of stevensjohnson syndrome and toxic. Vaccines, which undergo rigorous safety testing prior to. Stevensjohnson syndrome sjs and toxic epidermal necrolysis ten are. The main etiologic factors for both ssj and ten are sulphonamides and. Jan 01, 2003 background it has been proposed that fasfas ligand interaction was responsible for the apoptosis of epidermal cells in stevensjohnson syndrome sjs and toxic epidermal necrolysis ten and that high doses of intravenous immunoglobulin ivig could help patients by blocking the. A case of toxic epidermal necrolysislike skin lesions.
Toxic epidermal necrolysis ten clinical presentation. A total of 377 adult patients with sjsten between january 1, 2000 and june 1, 2015 were. Stevensjohnson syndrometoxic epidermal necrolysis sjsten is a severe skin reaction most often triggered by particular medications. Stevensjohnson syndrometoxic epidermal necrolysis can be prominent and, as at other. Review article toxic epidermal necrolysis management issues. Assessment of medication risks with emphasis on recently marketed drugs. Stevensjohnson syndrome is a medical emergency which is characterized by skin and mucosal reaction to the use of certain drugs. Toxic epidermal necrolysis ten is a rare, lifethreatening, usually druginduced, mucocutaneous disease characterized by extensive necrosis of the epidermis and widespread sloughing of the skin and mucosal surfaces. Review on toxic epidermal necrolysis toxic epidermal necrolysis ten is an acute, lifethreatening syndrome.
Morbidity and mortality of stevensjohnson syndrome and toxic epidermal necrolysis in united states adults. Stevensjohnson syndrome sjs and toxic epidermal necrolysis ten are caused by a reaction of the bodys own immune system. Loss of the skin leaves patients vulnerable to infections from fungi and bacteria, and can result in sepsis, the leading cause of death in the disease. Stevensjohnson syndrome sjs is a type of severe skin reaction. Toxic epidermal necrolysis ten management ahmed abdulghany 2. Stevensjohnson syndrome and toxic epidermal necrolysis in the. Both sjs and ten are hypersensitivity reactions, which means that the immune system reacts in a way that harms the body. Stevensjohnson syndrome sjs and toxic epidermal necrolysis ten are lifethreatening disorders.
Widespread fullthickness epidermal necrosis develops, producing erythema, and sloughing of the skin and mucosa, involving internal and external surfaces. Sixty years after its original description by sir alan lyell, epidermal necrolysis from stevensjohnson syndrome to toxic epidermal necrolysis seems finally amenable to a specific treatment in addition to essential symptomatic measures in specialized settings. Toxic epidermal necrolysis ten linkedin slideshare. Morbidity and mortality of stevensjohnson syndrome and. This was a retrospective study of sjs and ten in dr. Johnson syndrome is a medical emergency which is characterized by skin and mucosal reaction to the use of certain drugs.
They are differentiated based on the fraction of the body surface area affected. A case study of stevensjohnson syndrometoxic epidermal. They are characterized by mucocutaneous tenderness and. Update on pathobiology in stevensjohnson syndrome and. Recent evidence situates ten as the most severe form amongst a spectrum of severe epidermolytic adverse cutaneous drug reactions, which further include stevensjohnson syndrome sjs and the sjsten overlap disease 1. This multicenter retrospective study included patients with sjsten seen by inpatient consultative dermatologists at 18 academic medical centers in the united states. The patient was transferred to queen elizabeth hospital with a provisional diagnosis of toxic epidermal necrolysis after consultation with. Clinical classification of cases of toxic epidermal necrolysis, stevens johnson syndrome, and erythema multiforme. Use of intravenous immunoglobulin in children with stevensjohnson syndrome and toxic epidermal necrolysis. Introduction stevensjohnson syndrome sjs and toxic epidermal. Stevensjohnson syndrome toxic epidermal necrolysis. The mortality for toxic epidermal necrolysis is 2530%. Atypical stevenjohnson syndrome can occur due to various microorganisms and mycoplasma pneumoniae being one of them.
Prompt withdrawal of the culprit drug is the cornerstone of management including. Stevensjohnson syndrome sjs and toxic epidermal necrolysis ten are rare lifethreatening conditions almost exclusively attributed to drugs. Mycosplama pneumoniae infection may be involved in some cases of paediatric sjs. Both are rare, with ten and sjs affecting approximately 1or 21,000,000 annually, and are considered medical emergencies as they are potentially fatal. New evidence supporting cyclosporine efficacy in epidermal. Data of patients who diagnosed as sjs and ten, were obtained from medical records, including demographic information age, sex, relevant past medical history treatment, systemic corticosteroid treatment, and mortality. Management of stevensjohnson syndrome and toxic epidermal. Toxic epidermal necrolysis ten is a rare, lifethreatening adverse drug reaction with an estimated incidence of 0. A phenytoin induced toxic epidermal necrolysis ten a.
Although stevensjohnson syndrome and toxic epidermal necrolysis were once thought to be separate conditions, they are now considered part of a continuum. The main etiologic factors for both ssj and ten are sulphonamides and anticonvulsants. Publications home of jama and the specialty journals of the. Johnson syndrome can occur due to various microorganisms and mycoplasma pneumoniae being one of them. Toxic epidermal necrolysis and graftversushost reaction. Stevensjohnson syndrome and toxic epidermal necrolysis in. Toxic epidermal necrolysis syndrome falls in the spectrum of acute idiosyncratic bullous disorders with medications being the major aetiological factor. Pdf toxic epidermal necrolysis induced by phenytoin and. Stevensjohnson syndrometoxic epidermal necrolysis sjsten and erythema multiforme em are immunologicallymediated dermatological disorders commonly triggered by drug exposure andor other external agents. We aimed to characterise sjsten and emdrugrelated hospitalisations in a nationwide administrative database, focusing on demographic and clinical. Dec 16, 2010 toxic epidermal necrolysis ten and stevens johnson syndrome sjs are severe adverse cutaneous drug reactions that predominantly involve the skin and mucous membranes.
Medicines will help decrease pain or itching, or prevent an infection. Sepsis is the usual cause of mortality but much of the pathophysiologic process results from an outpouring of cytokines and matrixmetal. History of adverse drug reactions is an important risk factor for the development of hypersensibility to medication. Stevensjohnson syndrome sjs and toxic epidermal necrolysis ten are severe mucocutaneous reactions, most commonly triggered by medications, characterized by extensive necrosis and detachment of the epidermis. It is characterized by extensive erythema, cutaneous tenderness, and an exfoliation of. Stevensjohnson syndrome knowledge for medical students.
Rapidly developing toxic epidermal necrolysis hindawi. Dec 12, 2012 toxic epidermal necrolysis syndrome falls in the spectrum of acute idiosyncratic bullous disorders with medications being the major aetiological factor. Then the skin changes begin, with a flat red rash on the face, neck, and trunk, often. Toxic epidermal necrolysis ten is a rare, acute, and lifethreatening mucocutaneous disease that is usually drug related. Toxic epidermal necrolysis induced by phenytoin and whole brain radiotherapy. Stevensjohnson syndrome sjs and toxic epidermal necrolysis ten may be associated with a preceding history of medication use, most commonly anticonvulsants, antibiotics, and nonsteroidal antiinflammatory drugs.
Background stevensjohnson syndrome sjs and toxic epidermal necrolysis ten are rare but severe cutaneous drug reactions. Publications home of jama and the specialty journals of. Objective we compared the treatments given to and the clinical outcomes of 39 cases of sjs and 48 cases of ten seen at a single. By one commonly accepted definition, changes affect 30% of body surface area in ten. The usage of anti convulsants like carbamazepine, phenytoin, lamotrigine, phenobarbital, fosphenytoin are associated with high risk for occurrence of ten. Toxic epidermal necrolysis ten is a rare, potentially life threatening dermatological condition that is usually induced by medications. Iron complex, dabigatran and toxic epidermal necrolysis. Stevensjohnson syndrome and toxic epidermal necrolysis are among the most concerning drug reactions affecting adults and children. Fatal case of cephalexininduced toxic epidermal necrolysis. The incidence in children is lower than in adults and has a better outcome. Stevensjohnson syndrome, toxic epidermal necrolysis, dermatologic. Allopurinol, aromatic anticonvulsants, antibiotics, lamotrigine and oxicam nsaids 3. Based on histopathology, most clinicians consider stevensjohnson syndrome sjs and toxic epidermal necrolysis ten as different severities of the same potentially fatal mucocutaneous disease within the spectrum of erythema multiforme major emm. Once thought to be idiosyncratic and unpredictable, there has been significant progress made in the understanding of the pathomechanism and pharmacogenetics of such reactions.
Stevensjohnson syndrome sjstoxic epidermal necrolysis ten is a lifethreatening hypersensitivity reaction which is usually triggered by medications and carries a high mortality. Toxic epidermal necrolysis ten and stevenjohnson syndrome sjs are characterized by extensive necrosis and cleavage of the epidermis from the dermis akin to a superficial or partial thickness burn. Pdf toxic epidermal necrolysis ten and stevensjohnson. Although the overall mortality has reduced substantially after the introduction of several strategies, such as prompt withdrawal of the causal drug and management of the patients in an intensive care or burn unit, these conditions continue to be associated with. Hasan sadikin general hospital bandung, indonesia, between january 1, 2009 and december 31, 20. Stevensjohnson syndrome sjs and toxic epidermal necrolysis ten are uncommon, acute and potentially lifethreatening adverse cutaneous drug reactions, often related to drug use.
Jan 22, 2018 stevensjohnson syndrometoxic epidermal necrolysis sjsten and erythema multiforme em are immunologicallymediated dermatological disorders commonly triggered by drug exposure andor other external agents. Morbidity and mortality of stevensjohnson syndrome and toxic. The stevensjohnson syndrome sjs and toxic epidermal necrolysis ten. The patient had stated that the lesions were not there when he went to bed the night before and they were noticed by him first thing in the morning. In this second of three articles, we will examine the toxicology clues revealed by the skin. Stevensjohnson syndrome and toxic epidermal necrolysis. Sjs is a more severe form of erythema multiforme major and a less severe manifestation of toxic epidermal necrolysis ten. Both diseases present as an inflammatory cellpoor necrotic reaction. Diagnosis of the poisoned patient can often be accomplished before toxicologic laboratory tests by obtaining a detailed history and directed physical exam. Background it has been proposed that fasfas ligand interaction was responsible for the apoptosis of epidermal cells in stevensjohnson syndrome sjs and toxic epidermal necrolysis ten and that high doses of intravenous immunoglobulin ivig could help patients by blocking the apoptosis objective to study the effects of ivig on sjs and ten. Stevensjohnson syndrome sjs and its more severe variant, toxic epidermal necrolysis ten, are inflammatory disorders of the skin and mucous membranes that are characterized by acute, lifethreatening blistering and necrosis.
Dec 20, 2018 toxic epidermal necrolysis ten is a potentially lifethreatening dermatologic disorder characterized by widespread erythema, necrosis, and bullous detachment of the epidermis and mucous membranes, resulting in exfoliation and possible sepsis andor death see the image below. We aimed to characterise sjsten and emdrugrelated hospitalisations in a nationwide administrative database, focusing on demographic and clinical characteristics, and in the most. Our study objective was to describe the incidence, costs of care, length of stay, comorbidities, and mortality of sjs and ten in us adults. Stevensjohnson syndrometoxic epidermal necrolysis sjsten is a rare, severe mucocutaneous reaction with few large cohorts reported. So far, there are no serum biomarkers and no immunohistochemical criteria distinguishing with confidence and specificity the skin lesions of ten and gvhr. Part 1 dealt with clues found in eyes, hair, nails, and tongue, and part 3 will be. Mucous membranes are affected in over 90 percent of patients, usually at two or more distinct sites ocular, oral, and genital. Toxic epidermal necrolysis, skin detachment, keratinocyte cell death, stevensjohnson syndrome. Alternative medicine can produce cutaneous severity reactions. They are characterized by mucocutaneous tenderness and typically. Stevensjohnson syndrometoxic epidermal necrolysis and. Toxic epidermal necrolysis ten is a potentially lifethreatening dermatologic disorder characterized by widespread erythema, necrosis, and bullous detachment of the epidermis and mucous membranes, resulting in exfoliation and possible sepsis andor death see the image below.
Optimal therapy for sjs and ten is a controversial issue. Johnson syndrome and toxic epidermal necrolysis with. They are the result of extensive death of keratinocytes, which leads to the separation of areas of skin in the dermal epidermal junction, producing the appearance. Druginduced toxic epidermal necrolysis ten and acute cutaneous graftversushost reaction gvhr under immunopreventive therapy share some histopathological resemblance. The patient presents weeks after exposure to a medication with fever and other flulike symptoms. Toxic epidermal necrolysis discharge care what you. Toxic epidermal necrolysis ten is a rare, lifethreatening.
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