Materiały źródłowe

• #1 Stevens-Johnson Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK459323/

  Stevens-Johnson syndrome/toxic epidermal necrolysis is a rare and unpredictable reaction to medication that involves drug-specific CD8+ cytotoxic lymphocytes, the Fas-Fas ligand (FasL) pathway of apoptosis, and granule-mediated exocytosis and tumor necrosis factor-alfa (TNFalpha)/death receptor pathway. […] Medications are causative in over 80% of cases. […] Current theories address the following mechanisms, among others. […] Granulysin, found in the cytotoxic granules, is the main cause of keratinocyte apoptosis. […] FasFasL, expressed on the activated cytotoxic T cells, can also destroy keratinocytes via the production of intracellular caspases. […] Cytotoxic T cells exocytose perforin and granzyme B, which create channels in the target cell membrane activating the caspases. […] TNFalpha may cause apoptosis or protect from it. […] Nitrous oxide (NO) induced by TNFalpha and interferon (IFN)alpha may stimulate caspases.

• #2 Stevens-Johnson Syndrome (SJS): Causes, Rash & Treatments

  https://my.clevelandclinic.org/health/diseases/17656-stevens-johnson-syndrome

  Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are serious skin conditions that cause your skin to develop rashes, blisters, and then peel. […] Many cases of SJS happen in children and adults younger than 30 years old, but also occur in others, especially the elderly. More cases of SJS occur in females than males. Infections, like pneumonia, are the most likely cause of SJS in children, whereas medications are the most likely cause of SJS/TEN in adults. […] A combination of factors is likely involved in developing these disorders, including a genetic bias. Environmental factors might cause the gene to be triggered. One of these genetic factors include specific human leukocyte antigens (HLAs) that may increase ones risk of developing SJS or TEN. […] Causes of Stevens-Johnson syndrome include: Allergic reaction to a medication (most cases of SJS and almost all cases of TEN).

• #3 Toxic epidermal necrolysis and Stevens-Johnson syndrome | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-5-39

  Toxic epidermal necrolysis (TEN) and Stevens Johnson Syndrome (SJS) are severe adverse cutaneous drug reactions that predominantly involve the skin and mucous membranes. […] Drugs are assumed or identified as the main cause of SJS/TEN in most cases, but Mycoplasma pneumoniae and Herpes simplex virus infections are well documented causes alongside rare cases in which the aetiology remains unknown. […] Genetic susceptibility to SJS and TEN is likely as exemplified by the strong association observed in Han Chinese between a genetic marker, the human leukocyte antigen HLA-B*1502, and SJS induced by carbamazepine. […] A second strong association between HLA genotype and SJS/TEN has been reported for allopurinol. Indeed, 100% of Han Chinese patients with a severe adverse drug reaction to allopurinol were HLA-B*5801 positive.

• #4 Stevens-Johnson Syndrome: Causes, Diagnosis, and Treatment

  https://www.healthline.com/health/stevens-johnson-syndrome

  Stevens-Johnson syndrome (SJS) is a rare and extremely serious condition that causes your skin to come loose and detach. […] In more than 80 percent of cases, SJS is caused by an unpredictable adverse reaction to medication. Its less frequently caused by infections. […] SJS is most commonly caused by an immune response to certain medications. Its also possible for some infections to cause SJS. […] Some experts have suggested that a combination of medications and infections could together cause SJS, while in some cases, the trigger is never identified. […] While more than 100 medications have been associated with SJS, they generally fall into a handful of categories. SJS develops within a few days to 8 weeks after first taking the new medication. […] There are two main categories of infections that are associated with SJS: viral and bacterial.

• #5 Stevens-Johnson syndrome | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/stevens-johnson-syndrome

  Stevens-Johnson syndrome is a rare and unpredictable illness. Your health care provider may not be able to identify its exact cause, but usually the condition is triggered by medication, an infection or both. You may react to medication while you’re using it or up to two weeks after you’ve stopped using it. […] Drugs that can cause Stevens-Johnson syndrome include: Anti-gout medications, such as allopurinol; Medications to treat seizures and mental illness (anticonvulsants and antipsychotics); Antibacterial sulfonamides (including sulfasalazine); Nevirapine (Viramune, Viramune XR); Pain relievers, such as acetaminophen (Tylenol, others), ibuprofen (Advil, Motrin IB, others) and naproxen sodium (Aleve). […] Infections that can cause Stevens-Johnson syndrome include pneumonia and HIV.

• #6 Stevens Johnson Syndrome / Toxic Epidermal Necrolysis. SJS/TEN

  https://dermnetnz.org/topics/stevens-johnson-syndrome-toxic-epidermal-necrolysis

  Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are now believed to be variants of the same condition, distinct from erythema multiforme. SJS/TEN is a rare, acute, serious, and potentially fatal skin reaction in which there are sheet-like skin and mucosal loss. Using current definitions, it is nearly always caused by medications. […] SJS/TEN is a very rare complication of medication use (estimated at 12/million each year for SJS, and 0.41.2/million each year for TEN). […] More than 200 medications have been reported in association with SJS/TEN. […] SJS/TEN is a rare and unpredictable reaction to a medication. The mechanism has still not been understood and is complex. […] Drug-specific CD8+ cytotoxic lymphocytes can be detected in the early blister fluid. They have some natural killer cell activity and can probably kill keratinocytes by direct contact. Cytokines implicated include perforin/granzyme, granulysin, Fas-L and tumour necrosis factor alpha (TNF). […] There are probably two major pathways involved: […] Fas-Fas ligand pathway of apoptosis has been considered a pivotal step in the pathogenesis of TEN. […] Granule-mediated exocytosis via perforin and granzyme B resulting in cytotoxicity (cell death).

• #7 Stevens-Johnson Syndrome: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1197450-overview

  Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are characterized by significant keratinocyte apoptosis, epidermal necrosis, and separation between the dermis and epidermis. The apoptosis of keratinocytes is primarily driven by CD8 cytotoxic T cells and natural killer cells, which interact with human leukocyte antigens (HLA) and drug antigens. Genetic predispositions, particularly the strong association of HLA-B*15:02 with carbamazepine-induced SJS/TEN, have been identified in certain Asian populations, whereas no similar associations have been found in Japanese, Korean, or European populations. […] Various etiologic factors have been implicated as causes of Stevens-Johnson syndrome. Drugs most commonly are blamed. The 4 etiologic categories are as follows: Infectious, Drug-induced, Malignancy-related, Idiopathic. Stevens-Johnson syndrome is idiopathic in 25-50% of cases. Drugs and malignancies are most often implicated as the etiology in adults and elderly persons. Pediatric cases are related more often to infections.

• #8 Stevens-Johnson Syndrome (Causes, Symptoms, and Treatment)

  https://patient.info/doctor/stevens-johnson-syndrome

  Stevens-Johnson syndrome causes (aetiology)7 […] Approximately 75% of SJS/TEN are caused by medications and 25% by infections and other causes. […] […] Drugs most commonly associated with SJS and TEN […] Allopurinol […] Carbamazepine […] Sulfonamides: […] Trimethoprim-sulfamethoxazole. […] Sulfadiazine. […] Sulfasalazine. […] Antiviral agents: […] Nevirapine. […] Abacavir. […] Anticonvulsants: […] Phenobarbital. […] Phenytoin. […] Valproic acid. […] Lamotrigine. […] Others: […] Imidazole antifungal agents. […] Non-steroidal anti-inflammatory drugs (oxicam type such as meloxicam). […] Salicylates. […] Sertraline. […] Bupropion (rarely). […] Infection […] Viral: includes herpes simplex virus, Epstein-Barr virus, enteroviruses, HIV, Coxsackievirus, influenza, hepatitis, mumps, lymphogranuloma venereum, rickettsia and variola.

• #9 Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) – Dermatologic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/dermatologic-disorders/hypersensitivity-and-reactive-skin-disorders/stevens-johnson-syndrome-sjs-and-toxic-epidermal-necrolysis-ten

  Stevens-Johnson syndrome and toxic epidermal necrolysis are severe cutaneous hypersensitivity reactions. Medications, especially sulfa drugs, antiseizure medications, and antibiotics, are the most common causes. […] Medications precipitate most cases of SJS and TEN. The most common medication causes include sulfa drugs (eg, sulfasalazine), other antibiotics (eg, aminopenicillins [usually ampicillin or amoxicillin], fluoroquinolones, cephalosporins), antiseizure medications (eg, phenytoin, carbamazepine, phenobarbital, valproic acid and its derivatives, lamotrigine), nonsteroidal anti-inflammatory drugs (eg, piroxicam, meloxicam), antiretroviral medications (eg, nevirapine), miscellaneous individual medications (eg, allopurinol, chlormezanone), and immune checkpoint inhibitors. […] Cases that are not caused by medications are attributed to infection (mostly with Mycoplasma pneumoniae), vaccination, and graft-vs-host disease. […] In children with Stevens-Johnson syndrome, an infection is the most likely cause, and most of those are related to M. pneumoniae.

• #10 Stevens-Johnson syndrome/toxic epidermal necrolysis: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/stevens-johnson-syndrome-toxic-epidermal-necrolysis/

  Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a severe skin reaction most often triggered by particular medications. […] Several genetic changes have been found to increase the risk of SJS/TEN in response to triggering factors such as medications. […] The genetic variations most strongly associated with SJS/TEN occur in the HLA-B gene. […] Studies suggest that the HLA-B gene variations associated with SJS/TEN cause the immune system to react abnormally to certain medications. […] The drugs most frequently associated with SJS/TEN include several medications that are used to treat seizures (particularly carbamazepine, lamotrigine, and phenytoin); allopurinol, which is used to treat kidney stones and a form of arthritis called gout; a class of antibiotic drugs called sulfonamides; nevirapine, which is used to treat HIV infection; and a type of non-steroidal anti-inflammatory drugs (NSAIDs) called oxicams. […] Researchers suspect that a combination of infections and drugs could contribute to the disease in some individuals.

• #11 Stevens-Johnson syndrome

  https://www.nhs.uk/conditions/stevens-johnson-syndrome/

  Stevens-Johnson syndrome is a rare but serious skin reaction that’s usually caused by taking certain medicines. […] Stevens-Johnson syndrome is often caused by your body reacting to certain medicines, particularly some types of epilepsy medicines, antibiotics, and anti-inflammatory painkillers. […] Medicines commonly linked to Stevens-Johnson syndrome include allopurinol, carbamazepine, lamotrigine, nevirapine, oxicam anti-inflammatories, such as meloxicam and piroxicam, phenobarbital, phenytoin, sulfamethoxazole and other sulfa antibiotics, and sulfasalazine. […] In children, Stevens-Johnson syndrome can sometimes be caused by infections like cold or flu, cold sores, and glandular fever. […] If it’s thought Stevens-Johnson syndrome is being caused by a medicine you’re taking, the medicine will be stopped.

• #12 Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) – Skin Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/skin-disorders/hypersensitivity-and-reactive-skin-disorders/stevens-johnson-syndrome-sjs-and-toxic-epidermal-necrolysis-ten

  Stevens-Johnson syndrome and toxic epidermal necrolysis are commonly caused by medications or infections. […] Most cases of Stevens-Johnson syndrome and toxic epidermal necrolysis are caused by a reaction to a medication, most often sulfa and other antibiotics; antiseizure medications, such as phenytoin and carbamazepine; and certain other medications, such as piroxicam or allopurinol. […] Some cases are caused by a bacterial infection, vaccination, or graft-versus-host disease. In children with Stevens-Johnson syndrome, an infection is the most likely cause. […] These disorders occur in all age groups. They are more likely to occur in people with an abnormal immune system, such as those with a bone marrow transplant, in people with systemic lupus erythematosus, in people with other chronic joint and connective tissue diseases, or in people with human immunodeficiency virus (HIV) infection (particularly when people also have pneumonia caused by Pneumocystis jirovecii). The tendency to develop one of these disorders can run in families.

• #13 Stevens-Johnson Syndrome (Causes, Symptoms, and Treatment)

  https://patient.info/doctor/stevens-johnson-syndrome

  Stevens-Johnson syndrome causes (aetiology)7 […] Approximately 75% of SJS/TEN are caused by medications and 25% by infections and other causes. […] […] Drugs most commonly associated with SJS and TEN […] Allopurinol […] Carbamazepine […] Sulfonamides: […] Trimethoprim-sulfamethoxazole. […] Sulfadiazine. […] Sulfasalazine. […] Antiviral agents: […] Nevirapine. […] Abacavir. […] Anticonvulsants: […] Phenobarbital. […] Phenytoin. […] Valproic acid. […] Lamotrigine. […] Others: […] Imidazole antifungal agents. […] Non-steroidal anti-inflammatory drugs (oxicam type such as meloxicam). […] Salicylates. […] Sertraline. […] Bupropion (rarely). […] Infection […] Viral: includes herpes simplex virus, Epstein-Barr virus, enteroviruses, HIV, Coxsackievirus, influenza, hepatitis, mumps, lymphogranuloma venereum, rickettsia and variola.

• #14 What Are the Most Common Causes of Stevens–Johnson Syndrome?logo-32logo-40logo-60NEJM Journal WatchnejmJW_1L_RGB-b

  https://www.jwatch.org/na50905/2020/02/18/what-are-most-common-causes-stevens-johnson-syndrome

  Antiglaucoma eye drops, allopurinol, and antiseizure medications topped the list in Korea. […] Based on previous U.S. epidemiologic studies, drugs most commonly associated with Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) include allopurinol, anticonvulsants (e.g., lamotrigine, carbamazepine, phenytoin, phenobarbital), sulfamethoxazole, nevirapine, and oxicam nonsteroidal anti-inflammatory drugs. […] They determined that carbonic anhydrase inhibitors (used as antiglaucoma eye drops), allopurinol, carbamazepine, lamotrigine, fluoroquinolones, aminopenicillins, cephalosporins, and acetaminophen (in descending order) were the main causes of SJS/TEN. […] Interestingly, carbonic anhydrase inhibitors were the most common cause of SJS/TEN in South Korea, and sulfonamide antibiotics did not crack the top 10.

• #15 Toxic epidermal necrolysis and Stevens-Johnson syndrome | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-5-39

  Drug exposure and a resulting hypersensitivity reaction is the cause of the very large majority of cases of SJS/TEN. In absolute case numbers, allopurinol is the most common cause of SJS/TEN in Europe and Israel. […] The pathogenesis of SJS/TEN is not fully understood but is believed to be immune-mediated, as re-challenging an individual with the same drug can result in rapid recurrence of SJS/TEN. […] The histopathology of SJS/TEN lesions show that keratinocyte apoptosis followed by necrosis is the pathogenic basis of the widespread epidermal detachment observed in SJS/TEN.

• #16 Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) – Skin Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/skin-disorders/hypersensitivity-and-reactive-skin-disorders/stevens-johnson-syndrome-sjs-and-toxic-epidermal-necrolysis-ten

  Stevens-Johnson syndrome and toxic epidermal necrolysis are commonly caused by medications or infections. […] Most cases of Stevens-Johnson syndrome and toxic epidermal necrolysis are caused by a reaction to a medication, most often sulfa and other antibiotics; antiseizure medications, such as phenytoin and carbamazepine; and certain other medications, such as piroxicam or allopurinol. […] Some cases are caused by a bacterial infection, vaccination, or graft-versus-host disease. In children with Stevens-Johnson syndrome, an infection is the most likely cause. […] These disorders occur in all age groups. They are more likely to occur in people with an abnormal immune system, such as those with a bone marrow transplant, in people with systemic lupus erythematosus, in people with other chronic joint and connective tissue diseases, or in people with human immunodeficiency virus (HIV) infection (particularly when people also have pneumonia caused by Pneumocystis jirovecii). The tendency to develop one of these disorders can run in families.

• #17 Stevens-Johnson syndrome | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/stevens-johnson-syndrome

  Stevens-Johnson syndrome is a rare and unpredictable illness. Your health care provider may not be able to identify its exact cause, but usually the condition is triggered by medication, an infection or both. You may react to medication while you’re using it or up to two weeks after you’ve stopped using it. […] Drugs that can cause Stevens-Johnson syndrome include: Anti-gout medications, such as allopurinol; Medications to treat seizures and mental illness (anticonvulsants and antipsychotics); Antibacterial sulfonamides (including sulfasalazine); Nevirapine (Viramune, Viramune XR); Pain relievers, such as acetaminophen (Tylenol, others), ibuprofen (Advil, Motrin IB, others) and naproxen sodium (Aleve). […] Infections that can cause Stevens-Johnson syndrome include pneumonia and HIV.

• #18 Toxic Epidermal Necrolysis (TEN): Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/21616-toxic-epidermal-necrolysis-ten

  Toxic epidermal necrolysis (TEN) is a painful, life-threatening skin condition. It’s the result of a reaction to certain medications. […] Some people cannot normally break down certain medications, which can cause their body to develop Stevens-Johnson syndrome or toxic epidermal necrolysis. […] In most cases, experts think that certain medications trigger TEN syndrome. These medications may include: Allopurinol (drug used to treat kidney stones and gout), Anticonvulsants (group of drugs used to treat seizures), Anti-retroviral drugs used to treat HIV (especially NNRTIs like nevirapine, efavirenz, or etravirine), Oxicams (a group of non-steroidal anti-inflammatory drugs or NSAIDs), Sulfonamides (a type of antibiotic). […] There are some cases where the cause of TEN is not known.

• #19 Stevens-Johnson syndrome | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/stevens-johnson-syndrome

  Stevens-Johnson syndrome is a rare and unpredictable illness. Your health care provider may not be able to identify its exact cause, but usually the condition is triggered by medication, an infection or both. You may react to medication while you’re using it or up to two weeks after you’ve stopped using it. […] Drugs that can cause Stevens-Johnson syndrome include: Anti-gout medications, such as allopurinol; Medications to treat seizures and mental illness (anticonvulsants and antipsychotics); Antibacterial sulfonamides (including sulfasalazine); Nevirapine (Viramune, Viramune XR); Pain relievers, such as acetaminophen (Tylenol, others), ibuprofen (Advil, Motrin IB, others) and naproxen sodium (Aleve). […] Infections that can cause Stevens-Johnson syndrome include pneumonia and HIV.

• #20 Stevens-Johnson Syndrome: Symptoms and Causes

  https://resources.healthgrades.com/right-care/skin-hair-and-nails/stevens-johnson-syndrome

  Stevens-Johnson syndrome usually occurs when your body has an unusual reaction to certain medications. Your body releases a substance known as granulysin when you take these medications. […] Granulysin and other cytokines, such as IL13 and IL15, are believed to play a role in the progression of SJS. The causes of SJS are not fully understood. However, studies show elevated levels of granulysin in the skin samples of people with SJS. […] Typical causative medications include: NSAIDs, such as ibuprofen (Advil); anticonvulsants, such as gabapentin (Neurontin, Horizant, others), pregabalin (Lyrica), phenytoin (Dilantin), carbamazepine (Tegretol, Carbatrol); antipsychotics, such as risperidone (Risperdal) and quetiapine (Seroquel); antibiotics, such as penicillins, cephalosporins, and trimethoprim/sulfamethoxazole (Cotrim); nevirapine (Viramune), an HIV antiviral; allopurinol (Aloprim, others), a medication to treat gout. […] In rare cases, certain infections can also cause SJS. These infections include Mycoplasma pneumoniae infection. […] It is unclear why some people get SJS but others do not. However, experts believe genetic and nongenetic factors may play a role.

• #21

  https://link.springer.com/article/10.1007/s40257-024-00889-6

  More than 300 different drugs and supplements have been implicated. […] Most cases occur 4-28 days after initial exposure to the drug, though case reports for delayed-onset (6 months) SJS/TEN to lamotrigine and rapid-onset SJS/TEN to acetaminophen and penicillin (3 days) have been reported. […] Because of the importance of MHC to the pathogenesis of SJS/TEN and other drug hypersensitivity reactions, many of the genetic variants most closely linked to the condition are HLA alleles. […] In particular, HLA-B*15:02 is strongly associated with aromatic anticonvulsant-associated SJS/TEN, with reported odds ratios (OR) for carbamazepine ranging from 17 to 1357 in white and Asian populations. […] A few other genetic risk factors shed light on the pathogenesis of SJS/TEN polymorphisms in cytochrome P450 and other genes required for drug metabolism, and polymorphisms in the antigen presentation pathway. […] Therefore, further research is needed to better understand these risk factors and effectively use them in a clinical setting.

• #22 Stevens-Johnson Syndrome (Causes, Symptoms, and Treatment)

  https://patient.info/doctor/stevens-johnson-syndrome

  Stevens-Johnson syndrome causes (aetiology)7 […] Approximately 75% of SJS/TEN are caused by medications and 25% by infections and other causes. […] […] Drugs most commonly associated with SJS and TEN […] Allopurinol […] Carbamazepine […] Sulfonamides: […] Trimethoprim-sulfamethoxazole. […] Sulfadiazine. […] Sulfasalazine. […] Antiviral agents: […] Nevirapine. […] Abacavir. […] Anticonvulsants: […] Phenobarbital. […] Phenytoin. […] Valproic acid. […] Lamotrigine. […] Others: […] Imidazole antifungal agents. […] Non-steroidal anti-inflammatory drugs (oxicam type such as meloxicam). […] Salicylates. […] Sertraline. […] Bupropion (rarely). […] Infection […] Viral: includes herpes simplex virus, Epstein-Barr virus, enteroviruses, HIV, Coxsackievirus, influenza, hepatitis, mumps, lymphogranuloma venereum, rickettsia and variola.

• #23 Stevens-Johnson syndrome | Great Ormond Street Hospital

  https://www.gosh.nhs.uk/conditions-and-treatments/conditions-we-treat/stevens-johnson-syndrome/

  Stevens-Johnson syndrome is a rare condition arising from over-reaction of the immune system to a trigger such as a mild infection or a medicine, leading to blistering and peeling of the skin and surfaces of the eyes, mouth and throat. […] The most common triggers for Stevens-Johnson syndrome in children are infections, usually viral. Common infectious triggers include herpes, mumps, flu and the Epstein Barr virus. In adults, reactions to medicines, such as pain killers and antibiotics, are more common. In many cases, the trigger cannot be identified. […] Identifying the substance that triggered the overreaction will usually involve isolating an infection from a blood or sputum sample. All medicines may be stopped to identify which, if any, triggered the condition. In many cases, however, the cause cannot be identified.

• #24 Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) – Skin Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/skin-disorders/hypersensitivity-and-reactive-skin-disorders/stevens-johnson-syndrome-sjs-and-toxic-epidermal-necrolysis-ten

  Stevens-Johnson syndrome and toxic epidermal necrolysis are commonly caused by medications or infections. […] Most cases of Stevens-Johnson syndrome and toxic epidermal necrolysis are caused by a reaction to a medication, most often sulfa and other antibiotics; antiseizure medications, such as phenytoin and carbamazepine; and certain other medications, such as piroxicam or allopurinol. […] Some cases are caused by a bacterial infection, vaccination, or graft-versus-host disease. In children with Stevens-Johnson syndrome, an infection is the most likely cause. […] These disorders occur in all age groups. They are more likely to occur in people with an abnormal immune system, such as those with a bone marrow transplant, in people with systemic lupus erythematosus, in people with other chronic joint and connective tissue diseases, or in people with human immunodeficiency virus (HIV) infection (particularly when people also have pneumonia caused by Pneumocystis jirovecii). The tendency to develop one of these disorders can run in families.

• #25 Stevens–Johnson syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Stevens%E2%80%93Johnson_syndrome

  A leading cause appears to be the use of antibiotics, particularly sulfa drugs. […] Between 100 and 200 different drugs may be associated with SJS. […] The second most common cause of SJS and TEN is infection, particularly in children. […] This includes upper respiratory infections, otitis media, pharyngitis, and Epstein-Barr virus, Mycoplasma pneumoniae and cytomegalovirus infections. […] Viral diseases reported to cause SJS include: herpes simplex virus (possibly; is debated), AIDS, coxsackievirus, influenza, hepatitis, and mumps. […] In pediatric cases, Epstein-Barr virus and enteroviruses have been associated with SJS. […] Recent upper respiratory tract infections have been reported by more than half of patients with SJS. […] Bacterial infections linked to SJS include group A beta-hemolytic streptococci, diphtheria, brucellosis, lymphogranuloma venereum, mycobacteria, Mycoplasma pneumoniae, rickettsial infections, tularemia, and typhoid. […] Fungal infections with coccidioidomycosis, dermatophytosis and histoplasmosis are also considered possible causes. […] Malaria and trichomoniasis, protozoal infections, have also been reported as causes.

• #26 SciELO Brazil – Stevens-Johnson syndrome and toxic epidermal necrolysis: a review Stevens-Johnson syndrome and toxic epidermal necrolysis: a review

  https://www.scielo.br/j/ramb/a/D75h5BfLmz5Tw5JnMTXtPjF/

  The reported viral diseases include herpes simplex virus (HSV), HIV, coxsackievirus, influenza, hepatitis, lymphogranuloma venereum, and smallpox. Bacterial agents include group A beta-hemolytic streptococcus, the bacilli of diphtheria, brucellosis, typhoid fever and tularemia, mycobacteria and mycoplasma. Fungal causes include paracoccidioidomycosis, dermatophytosis and histoplasmosis. Protozoan parasites malaria and trichomonas were also related. In children, enteroviruses and Epstein-Barr virus are possible causative agents. Carcinomas and lymphomas are also associated. Notwithstanding the different known etiologies, SJS is idiopathic in 25 to 50% of cases.

• #27 Stevens-Johnson Syndrome – EyeWiki

  https://eyewiki.org/Stevens-Johnson_Syndrome

  Stevens-Johnson syndrome (SJS) is a dermatologic emergency, characterized by the presence of epidermal and mucosal bullous lesions involving less than 10% of the total body surface area (TBSA). […] Up to 75% of cases are attributed to delayed drug hypersensitivity reactions to a medication or medication metabolite. […] The remaining 25% of SJS cases not attributable to medication hypersensitivity are believed to be caused by an infectious source. […] The vast majority of these cases are due to Mycoplasma pneumoniae infection. […] The mechanism for M. pneumoniae-induced SJS remains unclear. […] It has been discovered that specific HLA subtypes carry an increased risk for development of SJS in various populations after exposure to certain classes of medications. […] The incidence of SJS was found to be 100 times higher in individuals infected with HIV relative to the general population. […] Rapid introduction of high dosages of medications associated with SJS further increases a patients risk of developing SJS.

• #28 Stevens–Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) | SpringerLink

  https://link.springer.com/10.1007/978-3-319-74588-6_195

  A small selection of these medications causes about half of all cases of SJS and TEN oxicam NSAIDs; phenylbutzone; sulfonamides such as sulfamethoxazole, sulfadiazine, sulfapyridine, sulfadoxine, sulfasalazine; allopurinol; lamotrigine; nevirapine; phenytoin; and carbamazepine. […] Mycoplasma pneumoniae is the most common bacterial infection associated with the development of SJS and TEN. […] Patients with hematologic malignancies, and to a lesser extent nonhematologic malignancies, have a higher risk of developing SJS and TEN. […] There are rare cases in which vaccinations have been thought to lead to SJS and TEN, including the smallpox vaccination, the diphtheria-pertussis-tetanus (DPT) vaccination, Bacillus Calmette-Gurin (BCG) vaccination, and the measles-mumps-rubella (MMR) vaccination. […] The incidence of SJS and TEN is about one or two cases per one million people. […] The SCORTEN scale allows for estimation of risk of death.

• #29 Stevens-Johnson Syndrome (Causes, Symptoms, and Treatment)

  https://patient.info/doctor/stevens-johnson-syndrome

  Bacterial: includes Group A beta-haemolytic streptococcus, diphtheria, brucellosis, mycobacteria, Mycoplasma pneumoniae, tularaemia and typhoid. […] Fungal: includes coccidioidomycosis, dermatophytosis and histoplasmosis. […] Protozoal: malaria and trichomoniasis. […] Immunisation […] Associated with immunisation – eg, measles, hepatitis B.

• #30 Stevens-Johnson Syndrome (Causes, Symptoms, and Treatment)

  https://patient.info/doctor/stevens-johnson-syndrome

  Bacterial: includes Group A beta-haemolytic streptococcus, diphtheria, brucellosis, mycobacteria, Mycoplasma pneumoniae, tularaemia and typhoid. […] Fungal: includes coccidioidomycosis, dermatophytosis and histoplasmosis. […] Protozoal: malaria and trichomoniasis. […] Immunisation […] Associated with immunisation – eg, measles, hepatitis B.

• #31 Stevens-Johnson syndrome | Great Ormond Street Hospital

  https://www.gosh.nhs.uk/conditions-and-treatments/conditions-we-treat/stevens-johnson-syndrome/

  Stevens-Johnson syndrome is a rare condition arising from over-reaction of the immune system to a trigger such as a mild infection or a medicine, leading to blistering and peeling of the skin and surfaces of the eyes, mouth and throat. […] The most common triggers for Stevens-Johnson syndrome in children are infections, usually viral. Common infectious triggers include herpes, mumps, flu and the Epstein Barr virus. In adults, reactions to medicines, such as pain killers and antibiotics, are more common. In many cases, the trigger cannot be identified. […] Identifying the substance that triggered the overreaction will usually involve isolating an infection from a blood or sputum sample. All medicines may be stopped to identify which, if any, triggered the condition. In many cases, however, the cause cannot be identified.

• #32 Stevens-Johnson Syndrome (SJS): Symptoms and Treatments

  https://www.allaboutvision.com/conditions/related/stevens-johnson-syndrome/

  Stevens-Johnson syndrome (SJS) is a rare and serious dermatological condition involving painful lesions and blisters on a persons skin. These abnormalities can also occur in mucous membranes, including the conjunctiva of the eye. […] The most common reason for Stevens-Johnson syndrome is an adverse reaction to medications. Experts believe these adverse reactions are responsible for up to 80 percent of SJS cases. […] Some types of infections can also cause Stevens-Johnson syndrome. Its most commonly caused by infections resulting from the bacterium Mycoplasma pneumoniae. Other infectious agents that may cause SJS include: Herpes simplex, Measles, Streptococcus, Adenovirus, Varicella zoster (chickenpox), Cytomegalovirus (CMV). […] Other factors, including chemotherapy, HIV and genetics, can also contribute to the development of SJS. Let your doctor know if anyone in your family has experienced SJS, as it raises your risk of developing the condition, too.

• #33 Stevens-Johnson syndrome | Great Ormond Street Hospital

  https://www.gosh.nhs.uk/conditions-and-treatments/conditions-we-treat/stevens-johnson-syndrome/

  Stevens-Johnson syndrome is a rare condition arising from over-reaction of the immune system to a trigger such as a mild infection or a medicine, leading to blistering and peeling of the skin and surfaces of the eyes, mouth and throat. […] The most common triggers for Stevens-Johnson syndrome in children are infections, usually viral. Common infectious triggers include herpes, mumps, flu and the Epstein Barr virus. In adults, reactions to medicines, such as pain killers and antibiotics, are more common. In many cases, the trigger cannot be identified. […] Identifying the substance that triggered the overreaction will usually involve isolating an infection from a blood or sputum sample. All medicines may be stopped to identify which, if any, triggered the condition. In many cases, however, the cause cannot be identified.

• #34 Stevens–Johnson syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Stevens%E2%80%93Johnson_syndrome

  A leading cause appears to be the use of antibiotics, particularly sulfa drugs. […] Between 100 and 200 different drugs may be associated with SJS. […] The second most common cause of SJS and TEN is infection, particularly in children. […] This includes upper respiratory infections, otitis media, pharyngitis, and Epstein-Barr virus, Mycoplasma pneumoniae and cytomegalovirus infections. […] Viral diseases reported to cause SJS include: herpes simplex virus (possibly; is debated), AIDS, coxsackievirus, influenza, hepatitis, and mumps. […] In pediatric cases, Epstein-Barr virus and enteroviruses have been associated with SJS. […] Recent upper respiratory tract infections have been reported by more than half of patients with SJS. […] Bacterial infections linked to SJS include group A beta-hemolytic streptococci, diphtheria, brucellosis, lymphogranuloma venereum, mycobacteria, Mycoplasma pneumoniae, rickettsial infections, tularemia, and typhoid. […] Fungal infections with coccidioidomycosis, dermatophytosis and histoplasmosis are also considered possible causes. […] Malaria and trichomoniasis, protozoal infections, have also been reported as causes.

• #35 A to Z: Stevens-Johnson Syndrome (for Parents) – Aetna Better Health of Kentucky (Medicaid)

  https://kidshealth.org/AetnaBetterHealthKentucky/en/parents/az-sjs.html

  Stevens-Johnson syndrome is a rare inflammatory disorder affecting the skin and mucous membranes. It’s usually triggered by exposure to an infection or a medication. […] In Stevens-Johnson syndrome, the immune system overreacts to a medication or infection. This causes flu-like symptoms, fever, blistering of the mucous membranes, and a red or purplish rash. […] Medications that are most likely to be involved in Stevens-Johnson syndrome include certain antibiotics, anti-seizure medications, and pain relievers. In children, infections with herpes viruses or Mycoplasma pneumoniae bacteria are often triggers. […] However, some cases happen without any known trigger.

• #36 Stevens–Johnson syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Stevens%E2%80%93Johnson_syndrome

  Stevens-Johnson syndrome (SJS) is a type of severe skin reaction. […] The most common cause is certain medications such as lamotrigine, carbamazepine, allopurinol, sulfonamide antibiotics and nevirapine. […] Other causes can include infections such as Mycoplasma pneumoniae and cytomegalovirus, or the cause may remain unknown. […] SJS is thought to arise from a disorder of the immune system. […] The immune reaction can be triggered by drugs or infections. […] Genetic factors are associated with a predisposition to SJS. […] The cause of SJS is unknown in one-quarter to one-half of cases. […] SJS, SJS/TEN, and TEN are considered a single disease with common causes and mechanisms. […] Although SJS can be caused by viral infections and malignancies, the main cause is medications.

• #37 Stevens-Johnson Syndrome – Causes, Symptoms, Diagnosis, Treatment, Prevention & Prognosis

  https://www.medindia.net/health/conditions/stevens-johnson-syndrome.htm

  Stevens-Johnson syndrome (SJS) is a rare but serious disorder that affects the skin and mucous membranes. It is an overreaction of the body’s immune system to a trigger like a medication or sometimes a mild infection. […] Causes of Stevens Johnson Syndrome include the following: Medications. This is the most common cause of SJS. The reaction in drug-induced SJS occurs within a week of starting the medication, though it may occur even a month or two later with medications like anticonvulsants. […] Infections that can predispose to SJS include the following: Viral infections – Infections with viruses such as mumps, influenza, herpes simplex, Epstein-Barr virus, hepatitis A, and HIV have been associated with SJS. […] Genetic factors – Genetic variations most likely associated with Stevens-Johnson syndrome occurs in the HLA-B gene. […] A weakened immune system as a result of HIV infection, autoimmune conditions, chemotherapy or organ transplants can predispose individuals to SJS. […] There is a risk of SJS recurring if the same medication or medications from the same group that had previously caused the reaction are taken.

• #38 Stevens–Johnson syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Stevens%E2%80%93Johnson_syndrome

  A leading cause appears to be the use of antibiotics, particularly sulfa drugs. […] Between 100 and 200 different drugs may be associated with SJS. […] The second most common cause of SJS and TEN is infection, particularly in children. […] This includes upper respiratory infections, otitis media, pharyngitis, and Epstein-Barr virus, Mycoplasma pneumoniae and cytomegalovirus infections. […] Viral diseases reported to cause SJS include: herpes simplex virus (possibly; is debated), AIDS, coxsackievirus, influenza, hepatitis, and mumps. […] In pediatric cases, Epstein-Barr virus and enteroviruses have been associated with SJS. […] Recent upper respiratory tract infections have been reported by more than half of patients with SJS. […] Bacterial infections linked to SJS include group A beta-hemolytic streptococci, diphtheria, brucellosis, lymphogranuloma venereum, mycobacteria, Mycoplasma pneumoniae, rickettsial infections, tularemia, and typhoid. […] Fungal infections with coccidioidomycosis, dermatophytosis and histoplasmosis are also considered possible causes. […] Malaria and trichomoniasis, protozoal infections, have also been reported as causes.

• #39 Stevens–Johnson syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Stevens%E2%80%93Johnson_syndrome

  A leading cause appears to be the use of antibiotics, particularly sulfa drugs. […] Between 100 and 200 different drugs may be associated with SJS. […] The second most common cause of SJS and TEN is infection, particularly in children. […] This includes upper respiratory infections, otitis media, pharyngitis, and Epstein-Barr virus, Mycoplasma pneumoniae and cytomegalovirus infections. […] Viral diseases reported to cause SJS include: herpes simplex virus (possibly; is debated), AIDS, coxsackievirus, influenza, hepatitis, and mumps. […] In pediatric cases, Epstein-Barr virus and enteroviruses have been associated with SJS. […] Recent upper respiratory tract infections have been reported by more than half of patients with SJS. […] Bacterial infections linked to SJS include group A beta-hemolytic streptococci, diphtheria, brucellosis, lymphogranuloma venereum, mycobacteria, Mycoplasma pneumoniae, rickettsial infections, tularemia, and typhoid. […] Fungal infections with coccidioidomycosis, dermatophytosis and histoplasmosis are also considered possible causes. […] Malaria and trichomoniasis, protozoal infections, have also been reported as causes.

• #40 Stevens-Johnson Syndrome (Causes, Symptoms, and Treatment)

  https://patient.info/doctor/stevens-johnson-syndrome

  Bacterial: includes Group A beta-haemolytic streptococcus, diphtheria, brucellosis, mycobacteria, Mycoplasma pneumoniae, tularaemia and typhoid. […] Fungal: includes coccidioidomycosis, dermatophytosis and histoplasmosis. […] Protozoal: malaria and trichomoniasis. […] Immunisation […] Associated with immunisation – eg, measles, hepatitis B.

• #41 Stevens-Johnson Syndrome (Causes, Symptoms, and Treatment)

  https://patient.info/doctor/stevens-johnson-syndrome

  Bacterial: includes Group A beta-haemolytic streptococcus, diphtheria, brucellosis, mycobacteria, Mycoplasma pneumoniae, tularaemia and typhoid. […] Fungal: includes coccidioidomycosis, dermatophytosis and histoplasmosis. […] Protozoal: malaria and trichomoniasis. […] Immunisation […] Associated with immunisation – eg, measles, hepatitis B.

• #42 Stevens–Johnson syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Stevens%E2%80%93Johnson_syndrome

  A leading cause appears to be the use of antibiotics, particularly sulfa drugs. […] Between 100 and 200 different drugs may be associated with SJS. […] The second most common cause of SJS and TEN is infection, particularly in children. […] This includes upper respiratory infections, otitis media, pharyngitis, and Epstein-Barr virus, Mycoplasma pneumoniae and cytomegalovirus infections. […] Viral diseases reported to cause SJS include: herpes simplex virus (possibly; is debated), AIDS, coxsackievirus, influenza, hepatitis, and mumps. […] In pediatric cases, Epstein-Barr virus and enteroviruses have been associated with SJS. […] Recent upper respiratory tract infections have been reported by more than half of patients with SJS. […] Bacterial infections linked to SJS include group A beta-hemolytic streptococci, diphtheria, brucellosis, lymphogranuloma venereum, mycobacteria, Mycoplasma pneumoniae, rickettsial infections, tularemia, and typhoid. […] Fungal infections with coccidioidomycosis, dermatophytosis and histoplasmosis are also considered possible causes. […] Malaria and trichomoniasis, protozoal infections, have also been reported as causes.

• #43 Cutaneous Adverse Reactions: Stevens-Johnson Syndrome

  https://www.uspharmacist.com/article/cutaneous-adverse-reactions-stevens-johnson-syndrome

  While causes of SJS include a variety of drugs, infections (viral, bacterial, fungal, protozoal), and malignancies (e.g., various carcinomas, lymphomas), up to 50% of cases have no identified etiology. […] More than 50% of patients with SJS report a recent upper respiratory tract infection. […] In adults and the elderly, drugs and malignancies are the etiologies most often implicated; infections most often cause SJS in pediatric patients. […] Most SJS reactions induced by anticonvulsants occur in the first 60 days of therapy. […] Exposure to corticosteroids has been associated with an increased rate of SJS occurrence. […] SJS occurs predominately in Caucasians; there is a male-to-female ratio of 2:1 and a propensity for the reaction to manifest in the early spring and winter. […] SJS can occur at any age; however, most patients are in the second to fourth decade of their lives. Cases have been reported in children as young as 3 months.

• #44 Stevens-Johnson Syndrome: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1197450-overview

  There is strong evidence for a genetic predisposition to severe cutaneous adverse drug reactions such as Stevens-Johnson syndrome. Carriage of the following human leukocyte antigens has been associated with increased risk: HLA-B*1502, HLA-B*5801, HLA-B*44, HLA-A29, HLA-B12, HLA-DR7, HLA-A2, HLA-B*5801, HLA-A*0206, HLA-DQB1*0601. Certain of these HLA alleles are associated with an increased probability of developing Stevens-Johnson syndrome upon exposure to specific drugs.

• #45 Azthena logo with the word Azthena

  https://www.news-medical.net/health/What-is-Stevens-Johnson-Syndrome.aspx

  Stevens-Johnson syndrome is frequently triggered by your body’s reaction to certain medications, including epilepsy medications, antibiotics, and anti-inflammatory pain relievers. […] The syndrome has an unpredictable reaction on medications, which involves drug-specific CD8+ cytotoxic lymphocytes, the Fas-Fas ligand (FasL) apoptotic pathway, granule-mediated exocytosis, and the tumor necrosis factor TNF-alpha receptor pathway. […] It can affect anybody with a genetic predisposition, regardless of age, gender, or ethnicity. Human leukocyte antigen (HLA) allotypes are genetic variables that raise the incidence of SJS. […] The HLA-B gene has the highest genetic variants related to SJS. […] According to research, HLA-B gene variants linked to SJS/TEN cause the immune system to react improperly to specific drugs.

• #46

  https://link.springer.com/article/10.1007/s40257-024-00889-6

  More than 300 different drugs and supplements have been implicated. […] Most cases occur 4-28 days after initial exposure to the drug, though case reports for delayed-onset (6 months) SJS/TEN to lamotrigine and rapid-onset SJS/TEN to acetaminophen and penicillin (3 days) have been reported. […] Because of the importance of MHC to the pathogenesis of SJS/TEN and other drug hypersensitivity reactions, many of the genetic variants most closely linked to the condition are HLA alleles. […] In particular, HLA-B*15:02 is strongly associated with aromatic anticonvulsant-associated SJS/TEN, with reported odds ratios (OR) for carbamazepine ranging from 17 to 1357 in white and Asian populations. […] A few other genetic risk factors shed light on the pathogenesis of SJS/TEN polymorphisms in cytochrome P450 and other genes required for drug metabolism, and polymorphisms in the antigen presentation pathway. […] Therefore, further research is needed to better understand these risk factors and effectively use them in a clinical setting.

• #47 Stevens-Johnson syndrome/toxic epidermal necrolysis: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/stevens-johnson-syndrome-toxic-epidermal-necrolysis/

  Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a severe skin reaction most often triggered by particular medications. […] Several genetic changes have been found to increase the risk of SJS/TEN in response to triggering factors such as medications. […] The genetic variations most strongly associated with SJS/TEN occur in the HLA-B gene. […] Studies suggest that the HLA-B gene variations associated with SJS/TEN cause the immune system to react abnormally to certain medications. […] The drugs most frequently associated with SJS/TEN include several medications that are used to treat seizures (particularly carbamazepine, lamotrigine, and phenytoin); allopurinol, which is used to treat kidney stones and a form of arthritis called gout; a class of antibiotic drugs called sulfonamides; nevirapine, which is used to treat HIV infection; and a type of non-steroidal anti-inflammatory drugs (NSAIDs) called oxicams. […] Researchers suspect that a combination of infections and drugs could contribute to the disease in some individuals.

• #48 Toxic epidermal necrolysis and Stevens-Johnson syndrome | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-5-39

  Toxic epidermal necrolysis (TEN) and Stevens Johnson Syndrome (SJS) are severe adverse cutaneous drug reactions that predominantly involve the skin and mucous membranes. […] Drugs are assumed or identified as the main cause of SJS/TEN in most cases, but Mycoplasma pneumoniae and Herpes simplex virus infections are well documented causes alongside rare cases in which the aetiology remains unknown. […] Genetic susceptibility to SJS and TEN is likely as exemplified by the strong association observed in Han Chinese between a genetic marker, the human leukocyte antigen HLA-B*1502, and SJS induced by carbamazepine. […] A second strong association between HLA genotype and SJS/TEN has been reported for allopurinol. Indeed, 100% of Han Chinese patients with a severe adverse drug reaction to allopurinol were HLA-B*5801 positive.

• #49 Stevens-Johnson Syndrome – Richard Harris Law Firm

  https://richardharrislaw.com/dangerous-drugs-products/stevens-johnson-syndrome/

  Stevens-Johnson Syndrome (SJS) is a condition that results in severe skin reactions, including rashes and blisters that can progress to third-degree burn-like intensity. SJS may result from many causes, though adverse drug reactions are often reported in conjunction with the condition. […] SJS has been documented as a side effect of, or reaction to: antibiotics, anti-convulsants (such as Dilantin), barbiturates, malignant diseases, nonsteroidal anti-inflammatory drugs (NSAIDs), both prescription and over the counter, sulfa or sulfa-containing drugs, viral or bacterial infections. […] Between 25 percent and 50 percent of all cases of SJS are reportedly idiopathic, that is, arising spontaneously with no apparent origin or cause. […] A recent study published in the Archives of Dermatology found that people of Asian descent are more susceptible to SJS reactions when treated with a certain epilepsy drug known as carbamazepine. The study found that a gene variant known as HLA-B*1502, which is present in about 10 percent of people of Han Chinese descent, is associated with increased incidences of SJS/TEN.

• #50 Toxic epidermal necrolysis and Stevens-Johnson syndrome | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-5-39

  Toxic epidermal necrolysis (TEN) and Stevens Johnson Syndrome (SJS) are severe adverse cutaneous drug reactions that predominantly involve the skin and mucous membranes. […] Drugs are assumed or identified as the main cause of SJS/TEN in most cases, but Mycoplasma pneumoniae and Herpes simplex virus infections are well documented causes alongside rare cases in which the aetiology remains unknown. […] Genetic susceptibility to SJS and TEN is likely as exemplified by the strong association observed in Han Chinese between a genetic marker, the human leukocyte antigen HLA-B*1502, and SJS induced by carbamazepine. […] A second strong association between HLA genotype and SJS/TEN has been reported for allopurinol. Indeed, 100% of Han Chinese patients with a severe adverse drug reaction to allopurinol were HLA-B*5801 positive.

• #51 Toxic epidermal necrolysis – Wikipedia

  https://en.wikipedia.org/wiki/Toxic_epidermal_necrolysis

  Toxic epidermal necrolysis (TEN), also known as Lyell’s syndrome, is a type of severe skin reaction. […] The most common cause is certain medications such as lamotrigine, carbamazepine, allopurinol, sulfonamide antibiotics, and nevirapine. […] Other causes can include infections such as Mycoplasma pneumoniae and cytomegalovirus or the cause may remain unknown. […] Drug reactions have been reported to cause 80-95% of TEN cases. […] The drugs most often implicated in TEN are: antibiotics, nonsteroidal anti-inflammatory drugs, allopurinol, antimetabolites, antiretroviral drugs, corticosteroids, anxiolytics, and anticonvulsants. […] TEN has also been reported to result from infection with Mycoplasma pneumoniae or dengue virus. […] HIV-positive individuals have 1000 times the risk of developing SJS/TEN compared to the general population. […] Certain genetic factors are associated with increased risk of TEN. For example, certain HLA-types such as, HLA-B*1502, HLA-A*3101, HLA-B*5801, and HLA-B*57:01 have been seen to be linked with TEN development when exposed to specific drugs.

• #52 Stevens-Johnson Syndrome: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1197450-overview

  There is strong evidence for a genetic predisposition to severe cutaneous adverse drug reactions such as Stevens-Johnson syndrome. Carriage of the following human leukocyte antigens has been associated with increased risk: HLA-B*1502, HLA-B*5801, HLA-B*44, HLA-A29, HLA-B12, HLA-DR7, HLA-A2, HLA-B*5801, HLA-A*0206, HLA-DQB1*0601. Certain of these HLA alleles are associated with an increased probability of developing Stevens-Johnson syndrome upon exposure to specific drugs.

• #53 Stevens-Johnson Syndrome | Causes | Symptoms | Treatment

  https://www.icliniq.com/articles/skin-care/stevens-johnson-syndrome

  Stevens-Johnson syndrome is a rare serious skin disease caused due to a reaction of medicines or infections. […] Stevens-Johnson syndrome is caused by medications causing an allergic reaction. The exact cause of this condition is not known. Researchers are still trying to identify the exact cause of this disease. […] More drugs are listed to be the cause of Stevens-Johnson syndrome. Some of them are: Medicines that are prescribed for the treatment of gout. Pain relievers such as Acetaminophen and Ibuprofen. Allopurinol drugs are given for the painful form of arthritis. Medicines that are used to treat seizures or any other mental illness. Certain antibiotics. […] Stevens-Johnson syndrome has the following risk factors: Viral infections such as herpes, viral pneumonia, hepatitis, and HIV (human immunodeficiency virus). Weakened Immune System: This may be the result of HIV or AIDS, lupus, or autoimmune conditions. A history of Stevens-Johnson syndrome. A family history of Stevens-Johnson syndrome. Certain genes are known to increase the risk of Stevens-Johnson syndrome. Chronic diseases of joints. Chronic diseases of the connective tissue.

• #54 Stevens-Johnson Syndrome: Causes, Diagnosis, and Treatment

  https://www.healthline.com/health/stevens-johnson-syndrome

  There have been reports of SJS linked to vaccinations, although it is extremely rare, totaling around a dozen known cases and associated with nearly as many different vaccines. There is no clear pattern or specific vaccine to be aware of. […] Certain medical conditions are risk factors for SJS, including being immunocompromised, having cancer, and having HIV, which increases the risk of SJS by 100 times. […] Your genes are also a risk factor for SJS. If a close family member has had SJS or TEN, your risk of SJS is increased. […] Variations of the gene HLA-B are associated with an increased risk of SJS and can sometimes be found in people of various ethnic identities. […] If youve had SJS or TEN before, you have a higher risk of another occurrence and should avoid known triggers.

• #55 Stevens-Johnson Syndrome – EyeWiki

  https://eyewiki.org/Stevens-Johnson_Syndrome

  Stevens-Johnson syndrome (SJS) is a dermatologic emergency, characterized by the presence of epidermal and mucosal bullous lesions involving less than 10% of the total body surface area (TBSA). […] Up to 75% of cases are attributed to delayed drug hypersensitivity reactions to a medication or medication metabolite. […] The remaining 25% of SJS cases not attributable to medication hypersensitivity are believed to be caused by an infectious source. […] The vast majority of these cases are due to Mycoplasma pneumoniae infection. […] The mechanism for M. pneumoniae-induced SJS remains unclear. […] It has been discovered that specific HLA subtypes carry an increased risk for development of SJS in various populations after exposure to certain classes of medications. […] The incidence of SJS was found to be 100 times higher in individuals infected with HIV relative to the general population. […] Rapid introduction of high dosages of medications associated with SJS further increases a patients risk of developing SJS.

• #56 Toxic epidermal necrolysis – Wikipedia

  https://en.wikipedia.org/wiki/Toxic_epidermal_necrolysis

  Toxic epidermal necrolysis (TEN), also known as Lyell’s syndrome, is a type of severe skin reaction. […] The most common cause is certain medications such as lamotrigine, carbamazepine, allopurinol, sulfonamide antibiotics, and nevirapine. […] Other causes can include infections such as Mycoplasma pneumoniae and cytomegalovirus or the cause may remain unknown. […] Drug reactions have been reported to cause 80-95% of TEN cases. […] The drugs most often implicated in TEN are: antibiotics, nonsteroidal anti-inflammatory drugs, allopurinol, antimetabolites, antiretroviral drugs, corticosteroids, anxiolytics, and anticonvulsants. […] TEN has also been reported to result from infection with Mycoplasma pneumoniae or dengue virus. […] HIV-positive individuals have 1000 times the risk of developing SJS/TEN compared to the general population. […] Certain genetic factors are associated with increased risk of TEN. For example, certain HLA-types such as, HLA-B*1502, HLA-A*3101, HLA-B*5801, and HLA-B*57:01 have been seen to be linked with TEN development when exposed to specific drugs.

• #57 Triggers for Stevens–Johnson syndrome / toxic epidermal necrolysis

  https://dermnetnz.org/topics/sjsten-triggers

  Medicines (listed alphabetically) Other causes […] This list of drugs known to cause SJS/TEN is not exclusive. […] Other causes […] Mycoplasma pneumoniae and cytomegalovirus infections […] Vaccinations […] Cancer, especially haematological cancers […] Systemic diseases […] Contrast medium […] External chemical exposure […] Herbal medicines […] Foods […] Bone marrow transplantation […] Radiotherapy.

• #58 Stevens-Johnson Syndrome: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1197450-overview

  Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are characterized by significant keratinocyte apoptosis, epidermal necrosis, and separation between the dermis and epidermis. The apoptosis of keratinocytes is primarily driven by CD8 cytotoxic T cells and natural killer cells, which interact with human leukocyte antigens (HLA) and drug antigens. Genetic predispositions, particularly the strong association of HLA-B*15:02 with carbamazepine-induced SJS/TEN, have been identified in certain Asian populations, whereas no similar associations have been found in Japanese, Korean, or European populations. […] Various etiologic factors have been implicated as causes of Stevens-Johnson syndrome. Drugs most commonly are blamed. The 4 etiologic categories are as follows: Infectious, Drug-induced, Malignancy-related, Idiopathic. Stevens-Johnson syndrome is idiopathic in 25-50% of cases. Drugs and malignancies are most often implicated as the etiology in adults and elderly persons. Pediatric cases are related more often to infections.

• #59 Stevens-Johnson Syndrome (SJS): Causes, Rash & Treatments

  https://my.clevelandclinic.org/health/diseases/17656-stevens-johnson-syndrome

  Medications most likely to cause Stevens-Johnson syndrome include: Antibacterial sulfa drugs. […] You are at greater risk of SJS if you have the following conditions: Bone marrow transplant. […] SJS may redevelop if you are exposed to the same medication known to have triggered the condition the first time. In such cases, the second episode is usually more severe than the first episode.

• #60 Triggers for Stevens–Johnson syndrome / toxic epidermal necrolysis

  https://dermnetnz.org/topics/sjsten-triggers

  Medicines (listed alphabetically) Other causes […] This list of drugs known to cause SJS/TEN is not exclusive. […] Other causes […] Mycoplasma pneumoniae and cytomegalovirus infections […] Vaccinations […] Cancer, especially haematological cancers […] Systemic diseases […] Contrast medium […] External chemical exposure […] Herbal medicines […] Foods […] Bone marrow transplantation […] Radiotherapy.

• #61 Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) – Skin Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/skin-disorders/hypersensitivity-and-reactive-skin-disorders/stevens-johnson-syndrome-sjs-and-toxic-epidermal-necrolysis-ten

  Stevens-Johnson syndrome and toxic epidermal necrolysis are commonly caused by medications or infections. […] Most cases of Stevens-Johnson syndrome and toxic epidermal necrolysis are caused by a reaction to a medication, most often sulfa and other antibiotics; antiseizure medications, such as phenytoin and carbamazepine; and certain other medications, such as piroxicam or allopurinol. […] Some cases are caused by a bacterial infection, vaccination, or graft-versus-host disease. In children with Stevens-Johnson syndrome, an infection is the most likely cause. […] These disorders occur in all age groups. They are more likely to occur in people with an abnormal immune system, such as those with a bone marrow transplant, in people with systemic lupus erythematosus, in people with other chronic joint and connective tissue diseases, or in people with human immunodeficiency virus (HIV) infection (particularly when people also have pneumonia caused by Pneumocystis jirovecii). The tendency to develop one of these disorders can run in families.

• #62 Quick Facts:Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) – MSD Manual Consumer Version

  https://www.msdmanuals.com/home/quick-facts-skin-disorders/hypersensitivity-and-reactive-skin-disorders/stevens-johnson-syndrome-sjs-and-toxic-epidermal-necrolysis-ten

  Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are forms of the same life-threatening skin disease. SJS and TEN are usually caused by a reaction to a medicine. […] SJS and TEN are most often caused by: A reaction to a medicine, most often sulfa and other antibiotics or medications for seizures (anticonvulsants). Less often, cases are caused by a bacterial infection or vaccination. […] SJS and TEN are more common in people who have: A bone marrow transplant, Lupus, HIV infection.

• #63 Stevens-Johnson Syndrome (SJS): Causes, Rash & Treatments

  https://my.clevelandclinic.org/health/diseases/17656-stevens-johnson-syndrome

  Medications most likely to cause Stevens-Johnson syndrome include: Antibacterial sulfa drugs. […] You are at greater risk of SJS if you have the following conditions: Bone marrow transplant. […] SJS may redevelop if you are exposed to the same medication known to have triggered the condition the first time. In such cases, the second episode is usually more severe than the first episode.

• #64 Stevens-Johnson Syndrome – Causes, Symptoms, Diagnosis, Treatment, Prevention & Prognosis

  https://www.medindia.net/health/conditions/stevens-johnson-syndrome.htm

  Stevens-Johnson syndrome (SJS) is a rare but serious disorder that affects the skin and mucous membranes. It is an overreaction of the body’s immune system to a trigger like a medication or sometimes a mild infection. […] Causes of Stevens Johnson Syndrome include the following: Medications. This is the most common cause of SJS. The reaction in drug-induced SJS occurs within a week of starting the medication, though it may occur even a month or two later with medications like anticonvulsants. […] Infections that can predispose to SJS include the following: Viral infections – Infections with viruses such as mumps, influenza, herpes simplex, Epstein-Barr virus, hepatitis A, and HIV have been associated with SJS. […] Genetic factors – Genetic variations most likely associated with Stevens-Johnson syndrome occurs in the HLA-B gene. […] A weakened immune system as a result of HIV infection, autoimmune conditions, chemotherapy or organ transplants can predispose individuals to SJS. […] There is a risk of SJS recurring if the same medication or medications from the same group that had previously caused the reaction are taken.

• #65 Stevens-Johnson Syndrome – EyeWiki

  https://eyewiki.org/Stevens-Johnson_Syndrome

  Stevens-Johnson syndrome (SJS) is a dermatologic emergency, characterized by the presence of epidermal and mucosal bullous lesions involving less than 10% of the total body surface area (TBSA). […] Up to 75% of cases are attributed to delayed drug hypersensitivity reactions to a medication or medication metabolite. […] The remaining 25% of SJS cases not attributable to medication hypersensitivity are believed to be caused by an infectious source. […] The vast majority of these cases are due to Mycoplasma pneumoniae infection. […] The mechanism for M. pneumoniae-induced SJS remains unclear. […] It has been discovered that specific HLA subtypes carry an increased risk for development of SJS in various populations after exposure to certain classes of medications. […] The incidence of SJS was found to be 100 times higher in individuals infected with HIV relative to the general population. […] Rapid introduction of high dosages of medications associated with SJS further increases a patients risk of developing SJS.

• #66 Toxic Epidermal Necrolysis in Children

  https://www.nationwidechildrens.org/conditions/health-library/toxic-epidermal-necrolysis-in-children

  Toxic epidermal necrolysis is a life-threatening skin disorder. It’s most often caused by a medicine reaction. […] The condition is most often triggered in the first 8 weeks of using a new medicine. It may be caused by medicines for: […] In rare cases, the condition may be caused by: […] A child is at risk if they have: […] Family history of toxic epidermal necrolysis or Stevens-Johnson syndrome.

• #67 Stevens-Johnson Syndrome (SJS): Symptoms and Treatments

  https://www.allaboutvision.com/conditions/related/stevens-johnson-syndrome/

  Stevens-Johnson syndrome (SJS) is a rare and serious dermatological condition involving painful lesions and blisters on a persons skin. These abnormalities can also occur in mucous membranes, including the conjunctiva of the eye. […] The most common reason for Stevens-Johnson syndrome is an adverse reaction to medications. Experts believe these adverse reactions are responsible for up to 80 percent of SJS cases. […] Some types of infections can also cause Stevens-Johnson syndrome. Its most commonly caused by infections resulting from the bacterium Mycoplasma pneumoniae. Other infectious agents that may cause SJS include: Herpes simplex, Measles, Streptococcus, Adenovirus, Varicella zoster (chickenpox), Cytomegalovirus (CMV). […] Other factors, including chemotherapy, HIV and genetics, can also contribute to the development of SJS. Let your doctor know if anyone in your family has experienced SJS, as it raises your risk of developing the condition, too.

• #68 Triggers for Stevens–Johnson syndrome / toxic epidermal necrolysis

  https://dermnetnz.org/topics/sjsten-triggers

  Medicines (listed alphabetically) Other causes […] This list of drugs known to cause SJS/TEN is not exclusive. […] Other causes […] Mycoplasma pneumoniae and cytomegalovirus infections […] Vaccinations […] Cancer, especially haematological cancers […] Systemic diseases […] Contrast medium […] External chemical exposure […] Herbal medicines […] Foods […] Bone marrow transplantation […] Radiotherapy.

• #69 Stevens-Johnson Syndrome: Causes, Diagnosis, and Treatment

  https://www.healthline.com/health/stevens-johnson-syndrome

  There have been reports of SJS linked to vaccinations, although it is extremely rare, totaling around a dozen known cases and associated with nearly as many different vaccines. There is no clear pattern or specific vaccine to be aware of. […] Certain medical conditions are risk factors for SJS, including being immunocompromised, having cancer, and having HIV, which increases the risk of SJS by 100 times. […] Your genes are also a risk factor for SJS. If a close family member has had SJS or TEN, your risk of SJS is increased. […] Variations of the gene HLA-B are associated with an increased risk of SJS and can sometimes be found in people of various ethnic identities. […] If youve had SJS or TEN before, you have a higher risk of another occurrence and should avoid known triggers.

• #70 Toxic Epidermal Necrolysis (TEN): Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/229698-overview

  Toxic epidermal necrolysis (TEN) is most commonly drug induced. […] However, the disorder occasionally has other potential etiologies, including infection including COVID-19, malignancy, and vaccinations, including following COVID-19 vaccination. […] TEN can be induced by drugs or infection or can be idiopathic. Medications are the major precipitating cause. Numerous medications have been implicated, including antibiotics, antiepileptic drugs, nonsteroidal anti-inflammatory drugs (NSAIDs), ampicillin, allopurinol, corticosteroids (topical and systemic), and the antiretroviral drugs nevirapine and abacavir. […] Infectious agents (ie, Mycoplasma pneumoniae, herpes virus, hepatitis A), immunizations (eg, meningococcal vaccine), and bone marrow or solid organ transplantation have also been associated with TEN.

• #71 Triggers for Stevens–Johnson syndrome / toxic epidermal necrolysis

  https://dermnetnz.org/topics/sjsten-triggers

  Medicines (listed alphabetically) Other causes […] This list of drugs known to cause SJS/TEN is not exclusive. […] Other causes […] Mycoplasma pneumoniae and cytomegalovirus infections […] Vaccinations […] Cancer, especially haematological cancers […] Systemic diseases […] Contrast medium […] External chemical exposure […] Herbal medicines […] Foods […] Bone marrow transplantation […] Radiotherapy.

• #72 Stevens-Johnson syndrome and toxic epidermal necrolysis – WikEM

  https://wikem.org/wiki/Stevens-Johnson_syndrome_and_toxic_epidermal_necrolysis

  Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are a spectrum of disease which ultimately results in blistering and peeling of the skin. […] SJS involves 10% of the skin body surface area. […] TEN involves 30% of the skin body surface area. […] SJS and TEN are considered true dermatologic emergencies. […] The most common cause overall is drugs. […] Many have been linked. Common offensive agents include: sulfa, quinolones, PCN, ASA, acetaminophen, carbamazepine, NSAIDs, phenytoin, corticosteroids, immunizations. […] High dose or rapid loading of allopurinol, lamotrigine. […] Malignancy – lymphoma, brain tumor treated with radiotherapy and antiepileptics. […] Idiopathic. […] Immunosuppression – HIV. […] Infectious – Mycoplasma pneumoniae. […] Autoimmune – SLE.

• #73 Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) – Dermatologic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/dermatologic-disorders/hypersensitivity-and-reactive-skin-disorders/stevens-johnson-syndrome-sjs-and-toxic-epidermal-necrolysis-ten

  Stevens-Johnson syndrome and toxic epidermal necrolysis are severe cutaneous hypersensitivity reactions. Medications, especially sulfa drugs, antiseizure medications, and antibiotics, are the most common causes. […] Medications precipitate most cases of SJS and TEN. The most common medication causes include sulfa drugs (eg, sulfasalazine), other antibiotics (eg, aminopenicillins [usually ampicillin or amoxicillin], fluoroquinolones, cephalosporins), antiseizure medications (eg, phenytoin, carbamazepine, phenobarbital, valproic acid and its derivatives, lamotrigine), nonsteroidal anti-inflammatory drugs (eg, piroxicam, meloxicam), antiretroviral medications (eg, nevirapine), miscellaneous individual medications (eg, allopurinol, chlormezanone), and immune checkpoint inhibitors. […] Cases that are not caused by medications are attributed to infection (mostly with Mycoplasma pneumoniae), vaccination, and graft-vs-host disease. […] In children with Stevens-Johnson syndrome, an infection is the most likely cause, and most of those are related to M. pneumoniae.

• #74 Triggers for Stevens–Johnson syndrome / toxic epidermal necrolysis

  https://dermnetnz.org/topics/sjsten-triggers

  Medicines (listed alphabetically) Other causes […] This list of drugs known to cause SJS/TEN is not exclusive. […] Other causes […] Mycoplasma pneumoniae and cytomegalovirus infections […] Vaccinations […] Cancer, especially haematological cancers […] Systemic diseases […] Contrast medium […] External chemical exposure […] Herbal medicines […] Foods […] Bone marrow transplantation […] Radiotherapy.

• #75 Triggers for Stevens–Johnson syndrome / toxic epidermal necrolysis

  https://dermnetnz.org/topics/sjsten-triggers

  Medicines (listed alphabetically) Other causes […] This list of drugs known to cause SJS/TEN is not exclusive. […] Other causes […] Mycoplasma pneumoniae and cytomegalovirus infections […] Vaccinations […] Cancer, especially haematological cancers […] Systemic diseases […] Contrast medium […] External chemical exposure […] Herbal medicines […] Foods […] Bone marrow transplantation […] Radiotherapy.

• #76 Stevens-Johnson Syndrome: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1197450-overview

  Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are characterized by significant keratinocyte apoptosis, epidermal necrosis, and separation between the dermis and epidermis. The apoptosis of keratinocytes is primarily driven by CD8 cytotoxic T cells and natural killer cells, which interact with human leukocyte antigens (HLA) and drug antigens. Genetic predispositions, particularly the strong association of HLA-B*15:02 with carbamazepine-induced SJS/TEN, have been identified in certain Asian populations, whereas no similar associations have been found in Japanese, Korean, or European populations. […] Various etiologic factors have been implicated as causes of Stevens-Johnson syndrome. Drugs most commonly are blamed. The 4 etiologic categories are as follows: Infectious, Drug-induced, Malignancy-related, Idiopathic. Stevens-Johnson syndrome is idiopathic in 25-50% of cases. Drugs and malignancies are most often implicated as the etiology in adults and elderly persons. Pediatric cases are related more often to infections.

• #77 Stevens–Johnson syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Stevens%E2%80%93Johnson_syndrome

  Stevens-Johnson syndrome (SJS) is a type of severe skin reaction. […] The most common cause is certain medications such as lamotrigine, carbamazepine, allopurinol, sulfonamide antibiotics and nevirapine. […] Other causes can include infections such as Mycoplasma pneumoniae and cytomegalovirus, or the cause may remain unknown. […] SJS is thought to arise from a disorder of the immune system. […] The immune reaction can be triggered by drugs or infections. […] Genetic factors are associated with a predisposition to SJS. […] The cause of SJS is unknown in one-quarter to one-half of cases. […] SJS, SJS/TEN, and TEN are considered a single disease with common causes and mechanisms. […] Although SJS can be caused by viral infections and malignancies, the main cause is medications.

• #78 Stevens-Johnson Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK459323/

  Stevens-Johnson syndrome/toxic epidermal necrolysis is a rare and unpredictable reaction to medication that involves drug-specific CD8+ cytotoxic lymphocytes, the Fas-Fas ligand (FasL) pathway of apoptosis, and granule-mediated exocytosis and tumor necrosis factor-alfa (TNFalpha)/death receptor pathway. […] Medications are causative in over 80% of cases. […] Current theories address the following mechanisms, among others. […] Granulysin, found in the cytotoxic granules, is the main cause of keratinocyte apoptosis. […] FasFasL, expressed on the activated cytotoxic T cells, can also destroy keratinocytes via the production of intracellular caspases. […] Cytotoxic T cells exocytose perforin and granzyme B, which create channels in the target cell membrane activating the caspases. […] TNFalpha may cause apoptosis or protect from it. […] Nitrous oxide (NO) induced by TNFalpha and interferon (IFN)alpha may stimulate caspases.

• #79 Stevens Johnson Syndrome / Toxic Epidermal Necrolysis. SJS/TEN

  https://dermnetnz.org/topics/stevens-johnson-syndrome-toxic-epidermal-necrolysis

  Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are now believed to be variants of the same condition, distinct from erythema multiforme. SJS/TEN is a rare, acute, serious, and potentially fatal skin reaction in which there are sheet-like skin and mucosal loss. Using current definitions, it is nearly always caused by medications. […] SJS/TEN is a very rare complication of medication use (estimated at 12/million each year for SJS, and 0.41.2/million each year for TEN). […] More than 200 medications have been reported in association with SJS/TEN. […] SJS/TEN is a rare and unpredictable reaction to a medication. The mechanism has still not been understood and is complex. […] Drug-specific CD8+ cytotoxic lymphocytes can be detected in the early blister fluid. They have some natural killer cell activity and can probably kill keratinocytes by direct contact. Cytokines implicated include perforin/granzyme, granulysin, Fas-L and tumour necrosis factor alpha (TNF). […] There are probably two major pathways involved: […] Fas-Fas ligand pathway of apoptosis has been considered a pivotal step in the pathogenesis of TEN. […] Granule-mediated exocytosis via perforin and granzyme B resulting in cytotoxicity (cell death).

• #80

  https://link.springer.com/article/10.1007/s40257-024-00889-6

  However, because SJS shares International Classification of Diseases, Tenth Revision (ICD-10) codes with erythema multiforme, incidence may be overestimated. […] Furthermore, 36-72% of patients initially diagnosed with SJS/TEN end up having their diagnosis reclassified into a different disease, also contributing to an overestimation of SJS/TEN incidence. […] The current understanding of SJS/TEN pathogenesis is summarized in Fig. 1. The key event is a tripartite interaction between a peptide presented by a major histocompatibility complex (MHC) on an antigen-presenting cell (APC) and a T cell receptor (TCR) expressed on a CD8+ (cytotoxic) T cell. […] Over 80% of cases are associated with medication exposure, particularly antimicrobials (sulfa antibiotics), antiepileptics, allopurinol, and nonsteroidal anti-inflammatory drugs (NSAIDs).

• #81 Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) – Dermatology – Diseases – McMaster Textbook of Internal Medicine

  https://empendium.com/mcmtextbook/chapter/B31.II.856.6.

  3. Environment: Immunocompromised patients, such as those with HIV/AIDS and those receiving chemotherapy or a combination of radiotherapy and anticonvulsants, are at increased risk. Infection by agents such as Mycoplasma pneumoniae, herpes simplex virus, and coxsackievirus A6 have also been described to affect susceptibility. […] The pathogenesis of the disease is complex. A delayed-type drug hypersensitivity reaction is considered the underlying cause. It involves a variety of cytotoxic T cells, natural killer (NK) cells, and several cytokines, which eventually lead to apoptosis and epidermal necrolysis.

• #82 Stevens-Johnson Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK459323/

  Stevens-Johnson syndrome/toxic epidermal necrolysis is a rare and unpredictable reaction to medication that involves drug-specific CD8+ cytotoxic lymphocytes, the Fas-Fas ligand (FasL) pathway of apoptosis, and granule-mediated exocytosis and tumor necrosis factor-alfa (TNFalpha)/death receptor pathway. […] Medications are causative in over 80% of cases. […] Current theories address the following mechanisms, among others. […] Granulysin, found in the cytotoxic granules, is the main cause of keratinocyte apoptosis. […] FasFasL, expressed on the activated cytotoxic T cells, can also destroy keratinocytes via the production of intracellular caspases. […] Cytotoxic T cells exocytose perforin and granzyme B, which create channels in the target cell membrane activating the caspases. […] TNFalpha may cause apoptosis or protect from it. […] Nitrous oxide (NO) induced by TNFalpha and interferon (IFN)alpha may stimulate caspases.

• #83 Stevens Johnson Syndrome / Toxic Epidermal Necrolysis. SJS/TEN

  https://dermnetnz.org/topics/stevens-johnson-syndrome-toxic-epidermal-necrolysis

  Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are now believed to be variants of the same condition, distinct from erythema multiforme. SJS/TEN is a rare, acute, serious, and potentially fatal skin reaction in which there are sheet-like skin and mucosal loss. Using current definitions, it is nearly always caused by medications. […] SJS/TEN is a very rare complication of medication use (estimated at 12/million each year for SJS, and 0.41.2/million each year for TEN). […] More than 200 medications have been reported in association with SJS/TEN. […] SJS/TEN is a rare and unpredictable reaction to a medication. The mechanism has still not been understood and is complex. […] Drug-specific CD8+ cytotoxic lymphocytes can be detected in the early blister fluid. They have some natural killer cell activity and can probably kill keratinocytes by direct contact. Cytokines implicated include perforin/granzyme, granulysin, Fas-L and tumour necrosis factor alpha (TNF). […] There are probably two major pathways involved: […] Fas-Fas ligand pathway of apoptosis has been considered a pivotal step in the pathogenesis of TEN. […] Granule-mediated exocytosis via perforin and granzyme B resulting in cytotoxicity (cell death).

• #84 Azthena logo with the word Azthena

  https://www.news-medical.net/health/What-is-Stevens-Johnson-Syndrome.aspx

  Stevens-Johnson syndrome is frequently triggered by your body’s reaction to certain medications, including epilepsy medications, antibiotics, and anti-inflammatory pain relievers. […] The syndrome has an unpredictable reaction on medications, which involves drug-specific CD8+ cytotoxic lymphocytes, the Fas-Fas ligand (FasL) apoptotic pathway, granule-mediated exocytosis, and the tumor necrosis factor TNF-alpha receptor pathway. […] It can affect anybody with a genetic predisposition, regardless of age, gender, or ethnicity. Human leukocyte antigen (HLA) allotypes are genetic variables that raise the incidence of SJS. […] The HLA-B gene has the highest genetic variants related to SJS. […] According to research, HLA-B gene variants linked to SJS/TEN cause the immune system to react improperly to specific drugs.

• #85 Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) – Dermatology – Diseases – McMaster Textbook of Internal Medicine

  https://empendium.com/mcmtextbook/chapter/B31.II.856.6.

  3. Environment: Immunocompromised patients, such as those with HIV/AIDS and those receiving chemotherapy or a combination of radiotherapy and anticonvulsants, are at increased risk. Infection by agents such as Mycoplasma pneumoniae, herpes simplex virus, and coxsackievirus A6 have also been described to affect susceptibility. […] The pathogenesis of the disease is complex. A delayed-type drug hypersensitivity reaction is considered the underlying cause. It involves a variety of cytotoxic T cells, natural killer (NK) cells, and several cytokines, which eventually lead to apoptosis and epidermal necrolysis.

• #86 Azthena logo with the word Azthena

  https://www.news-medical.net/health/What-is-Stevens-Johnson-Syndrome.aspx

  Stevens-Johnson syndrome is frequently triggered by your body’s reaction to certain medications, including epilepsy medications, antibiotics, and anti-inflammatory pain relievers. […] The syndrome has an unpredictable reaction on medications, which involves drug-specific CD8+ cytotoxic lymphocytes, the Fas-Fas ligand (FasL) apoptotic pathway, granule-mediated exocytosis, and the tumor necrosis factor TNF-alpha receptor pathway. […] It can affect anybody with a genetic predisposition, regardless of age, gender, or ethnicity. Human leukocyte antigen (HLA) allotypes are genetic variables that raise the incidence of SJS. […] The HLA-B gene has the highest genetic variants related to SJS. […] According to research, HLA-B gene variants linked to SJS/TEN cause the immune system to react improperly to specific drugs.

• #87

  https://link.springer.com/article/10.1007/s40257-024-00889-6

  However, because SJS shares International Classification of Diseases, Tenth Revision (ICD-10) codes with erythema multiforme, incidence may be overestimated. […] Furthermore, 36-72% of patients initially diagnosed with SJS/TEN end up having their diagnosis reclassified into a different disease, also contributing to an overestimation of SJS/TEN incidence. […] The current understanding of SJS/TEN pathogenesis is summarized in Fig. 1. The key event is a tripartite interaction between a peptide presented by a major histocompatibility complex (MHC) on an antigen-presenting cell (APC) and a T cell receptor (TCR) expressed on a CD8+ (cytotoxic) T cell. […] Over 80% of cases are associated with medication exposure, particularly antimicrobials (sulfa antibiotics), antiepileptics, allopurinol, and nonsteroidal anti-inflammatory drugs (NSAIDs).

• #88 Stevens Johnson Syndrome / Toxic Epidermal Necrolysis. SJS/TEN

  https://dermnetnz.org/topics/stevens-johnson-syndrome-toxic-epidermal-necrolysis

  Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are now believed to be variants of the same condition, distinct from erythema multiforme. SJS/TEN is a rare, acute, serious, and potentially fatal skin reaction in which there are sheet-like skin and mucosal loss. Using current definitions, it is nearly always caused by medications. […] SJS/TEN is a very rare complication of medication use (estimated at 12/million each year for SJS, and 0.41.2/million each year for TEN). […] More than 200 medications have been reported in association with SJS/TEN. […] SJS/TEN is a rare and unpredictable reaction to a medication. The mechanism has still not been understood and is complex. […] Drug-specific CD8+ cytotoxic lymphocytes can be detected in the early blister fluid. They have some natural killer cell activity and can probably kill keratinocytes by direct contact. Cytokines implicated include perforin/granzyme, granulysin, Fas-L and tumour necrosis factor alpha (TNF). […] There are probably two major pathways involved: […] Fas-Fas ligand pathway of apoptosis has been considered a pivotal step in the pathogenesis of TEN. […] Granule-mediated exocytosis via perforin and granzyme B resulting in cytotoxicity (cell death).

• #89 Stevens-Johnson Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK459323/

  Stevens-Johnson syndrome/toxic epidermal necrolysis is a rare and unpredictable reaction to medication that involves drug-specific CD8+ cytotoxic lymphocytes, the Fas-Fas ligand (FasL) pathway of apoptosis, and granule-mediated exocytosis and tumor necrosis factor-alfa (TNFalpha)/death receptor pathway. […] Medications are causative in over 80% of cases. […] Current theories address the following mechanisms, among others. […] Granulysin, found in the cytotoxic granules, is the main cause of keratinocyte apoptosis. […] FasFasL, expressed on the activated cytotoxic T cells, can also destroy keratinocytes via the production of intracellular caspases. […] Cytotoxic T cells exocytose perforin and granzyme B, which create channels in the target cell membrane activating the caspases. […] TNFalpha may cause apoptosis or protect from it. […] Nitrous oxide (NO) induced by TNFalpha and interferon (IFN)alpha may stimulate caspases.

• #90 Stevens-Johnson Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK459323/

  Stevens-Johnson syndrome/toxic epidermal necrolysis is a rare and unpredictable reaction to medication that involves drug-specific CD8+ cytotoxic lymphocytes, the Fas-Fas ligand (FasL) pathway of apoptosis, and granule-mediated exocytosis and tumor necrosis factor-alfa (TNFalpha)/death receptor pathway. […] Medications are causative in over 80% of cases. […] Current theories address the following mechanisms, among others. […] Granulysin, found in the cytotoxic granules, is the main cause of keratinocyte apoptosis. […] FasFasL, expressed on the activated cytotoxic T cells, can also destroy keratinocytes via the production of intracellular caspases. […] Cytotoxic T cells exocytose perforin and granzyme B, which create channels in the target cell membrane activating the caspases. […] TNFalpha may cause apoptosis or protect from it. […] Nitrous oxide (NO) induced by TNFalpha and interferon (IFN)alpha may stimulate caspases.

• #91 Stevens Johnson Syndrome / Toxic Epidermal Necrolysis. SJS/TEN

  https://dermnetnz.org/topics/stevens-johnson-syndrome-toxic-epidermal-necrolysis

  Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are now believed to be variants of the same condition, distinct from erythema multiforme. SJS/TEN is a rare, acute, serious, and potentially fatal skin reaction in which there are sheet-like skin and mucosal loss. Using current definitions, it is nearly always caused by medications. […] SJS/TEN is a very rare complication of medication use (estimated at 12/million each year for SJS, and 0.41.2/million each year for TEN). […] More than 200 medications have been reported in association with SJS/TEN. […] SJS/TEN is a rare and unpredictable reaction to a medication. The mechanism has still not been understood and is complex. […] Drug-specific CD8+ cytotoxic lymphocytes can be detected in the early blister fluid. They have some natural killer cell activity and can probably kill keratinocytes by direct contact. Cytokines implicated include perforin/granzyme, granulysin, Fas-L and tumour necrosis factor alpha (TNF). […] There are probably two major pathways involved: […] Fas-Fas ligand pathway of apoptosis has been considered a pivotal step in the pathogenesis of TEN. […] Granule-mediated exocytosis via perforin and granzyme B resulting in cytotoxicity (cell death).

• #92 Stevens-Johnson Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK459323/

  Stevens-Johnson syndrome/toxic epidermal necrolysis is a rare and unpredictable reaction to medication that involves drug-specific CD8+ cytotoxic lymphocytes, the Fas-Fas ligand (FasL) pathway of apoptosis, and granule-mediated exocytosis and tumor necrosis factor-alfa (TNFalpha)/death receptor pathway. […] Medications are causative in over 80% of cases. […] Current theories address the following mechanisms, among others. […] Granulysin, found in the cytotoxic granules, is the main cause of keratinocyte apoptosis. […] FasFasL, expressed on the activated cytotoxic T cells, can also destroy keratinocytes via the production of intracellular caspases. […] Cytotoxic T cells exocytose perforin and granzyme B, which create channels in the target cell membrane activating the caspases. […] TNFalpha may cause apoptosis or protect from it. […] Nitrous oxide (NO) induced by TNFalpha and interferon (IFN)alpha may stimulate caspases.

• #93 Stevens Johnson Syndrome / Toxic Epidermal Necrolysis. SJS/TEN

  https://dermnetnz.org/topics/stevens-johnson-syndrome-toxic-epidermal-necrolysis

  Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are now believed to be variants of the same condition, distinct from erythema multiforme. SJS/TEN is a rare, acute, serious, and potentially fatal skin reaction in which there are sheet-like skin and mucosal loss. Using current definitions, it is nearly always caused by medications. […] SJS/TEN is a very rare complication of medication use (estimated at 12/million each year for SJS, and 0.41.2/million each year for TEN). […] More than 200 medications have been reported in association with SJS/TEN. […] SJS/TEN is a rare and unpredictable reaction to a medication. The mechanism has still not been understood and is complex. […] Drug-specific CD8+ cytotoxic lymphocytes can be detected in the early blister fluid. They have some natural killer cell activity and can probably kill keratinocytes by direct contact. Cytokines implicated include perforin/granzyme, granulysin, Fas-L and tumour necrosis factor alpha (TNF). […] There are probably two major pathways involved: […] Fas-Fas ligand pathway of apoptosis has been considered a pivotal step in the pathogenesis of TEN. […] Granule-mediated exocytosis via perforin and granzyme B resulting in cytotoxicity (cell death).

• #94 Stevens-Johnson Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK459323/

  Stevens-Johnson syndrome/toxic epidermal necrolysis is a rare and unpredictable reaction to medication that involves drug-specific CD8+ cytotoxic lymphocytes, the Fas-Fas ligand (FasL) pathway of apoptosis, and granule-mediated exocytosis and tumor necrosis factor-alfa (TNFalpha)/death receptor pathway. […] Medications are causative in over 80% of cases. […] Current theories address the following mechanisms, among others. […] Granulysin, found in the cytotoxic granules, is the main cause of keratinocyte apoptosis. […] FasFasL, expressed on the activated cytotoxic T cells, can also destroy keratinocytes via the production of intracellular caspases. […] Cytotoxic T cells exocytose perforin and granzyme B, which create channels in the target cell membrane activating the caspases. […] TNFalpha may cause apoptosis or protect from it. […] Nitrous oxide (NO) induced by TNFalpha and interferon (IFN)alpha may stimulate caspases.

• #95 Stevens-Johnson Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK459323/

  Stevens-Johnson syndrome/toxic epidermal necrolysis is a rare and unpredictable reaction to medication that involves drug-specific CD8+ cytotoxic lymphocytes, the Fas-Fas ligand (FasL) pathway of apoptosis, and granule-mediated exocytosis and tumor necrosis factor-alfa (TNFalpha)/death receptor pathway. […] Medications are causative in over 80% of cases. […] Current theories address the following mechanisms, among others. […] Granulysin, found in the cytotoxic granules, is the main cause of keratinocyte apoptosis. […] FasFasL, expressed on the activated cytotoxic T cells, can also destroy keratinocytes via the production of intracellular caspases. […] Cytotoxic T cells exocytose perforin and granzyme B, which create channels in the target cell membrane activating the caspases. […] TNFalpha may cause apoptosis or protect from it. […] Nitrous oxide (NO) induced by TNFalpha and interferon (IFN)alpha may stimulate caspases.

• #96 Stevens-Johnson Syndrome: Symptoms and Causes

  https://resources.healthgrades.com/right-care/skin-hair-and-nails/stevens-johnson-syndrome

  Stevens-Johnson syndrome usually occurs when your body has an unusual reaction to certain medications. Your body releases a substance known as granulysin when you take these medications. […] Granulysin and other cytokines, such as IL13 and IL15, are believed to play a role in the progression of SJS. The causes of SJS are not fully understood. However, studies show elevated levels of granulysin in the skin samples of people with SJS. […] Typical causative medications include: NSAIDs, such as ibuprofen (Advil); anticonvulsants, such as gabapentin (Neurontin, Horizant, others), pregabalin (Lyrica), phenytoin (Dilantin), carbamazepine (Tegretol, Carbatrol); antipsychotics, such as risperidone (Risperdal) and quetiapine (Seroquel); antibiotics, such as penicillins, cephalosporins, and trimethoprim/sulfamethoxazole (Cotrim); nevirapine (Viramune), an HIV antiviral; allopurinol (Aloprim, others), a medication to treat gout. […] In rare cases, certain infections can also cause SJS. These infections include Mycoplasma pneumoniae infection. […] It is unclear why some people get SJS but others do not. However, experts believe genetic and nongenetic factors may play a role.

• #97 Stevens-Johnson Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK459323/

  Stevens-Johnson syndrome/toxic epidermal necrolysis is a rare and unpredictable reaction to medication that involves drug-specific CD8+ cytotoxic lymphocytes, the Fas-Fas ligand (FasL) pathway of apoptosis, and granule-mediated exocytosis and tumor necrosis factor-alfa (TNFalpha)/death receptor pathway. […] Medications are causative in over 80% of cases. […] Current theories address the following mechanisms, among others. […] Granulysin, found in the cytotoxic granules, is the main cause of keratinocyte apoptosis. […] FasFasL, expressed on the activated cytotoxic T cells, can also destroy keratinocytes via the production of intracellular caspases. […] Cytotoxic T cells exocytose perforin and granzyme B, which create channels in the target cell membrane activating the caspases. […] TNFalpha may cause apoptosis or protect from it. […] Nitrous oxide (NO) induced by TNFalpha and interferon (IFN)alpha may stimulate caspases.

• #98 Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) – Dermatology – Diseases – McMaster Textbook of Internal Medicine

  https://empendium.com/mcmtextbook/chapter/B31.II.856.6.

  Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening diseases characterized by mucocutaneous reactions, predominantly induced by drugs. SJS is idiopathic in 25% to 50% of cases. […] The etiology includes a combination of several known risk factors such as: […] 1. Drugs: The most commonly implicated are allopurinol, nonsteroidal anti-inflammatory drugs (NSAIDs), antibiotics (especially sulfonamides and penicillins), anticonvulsants (carbamazepine, lamotrigine, and phenytoin), and antiretrovirals. […] 2. Genetic predisposition: Genetic variants of cytochrome P450 2C (CYP2C) lead to decreased clearance of drugs (eg, anticonvulsants) and increased retention of active metabolites. SJS/TEN has been found to be related to the prevalence of susceptibility alleles in populations of specific ethnicities.

• #99

  https://link.springer.com/article/10.1007/s40257-024-00889-6

  More than 300 different drugs and supplements have been implicated. […] Most cases occur 4-28 days after initial exposure to the drug, though case reports for delayed-onset (6 months) SJS/TEN to lamotrigine and rapid-onset SJS/TEN to acetaminophen and penicillin (3 days) have been reported. […] Because of the importance of MHC to the pathogenesis of SJS/TEN and other drug hypersensitivity reactions, many of the genetic variants most closely linked to the condition are HLA alleles. […] In particular, HLA-B*15:02 is strongly associated with aromatic anticonvulsant-associated SJS/TEN, with reported odds ratios (OR) for carbamazepine ranging from 17 to 1357 in white and Asian populations. […] A few other genetic risk factors shed light on the pathogenesis of SJS/TEN polymorphisms in cytochrome P450 and other genes required for drug metabolism, and polymorphisms in the antigen presentation pathway. […] Therefore, further research is needed to better understand these risk factors and effectively use them in a clinical setting.

• #100 Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) – Dermatology – Diseases – McMaster Textbook of Internal Medicine

  https://empendium.com/mcmtextbook/chapter/B31.II.856.6.

  Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening diseases characterized by mucocutaneous reactions, predominantly induced by drugs. SJS is idiopathic in 25% to 50% of cases. […] The etiology includes a combination of several known risk factors such as: […] 1. Drugs: The most commonly implicated are allopurinol, nonsteroidal anti-inflammatory drugs (NSAIDs), antibiotics (especially sulfonamides and penicillins), anticonvulsants (carbamazepine, lamotrigine, and phenytoin), and antiretrovirals. […] 2. Genetic predisposition: Genetic variants of cytochrome P450 2C (CYP2C) lead to decreased clearance of drugs (eg, anticonvulsants) and increased retention of active metabolites. SJS/TEN has been found to be related to the prevalence of susceptibility alleles in populations of specific ethnicities.

• #101

  https://link.springer.com/article/10.1007/s40257-024-00889-6

  More than 300 different drugs and supplements have been implicated. […] Most cases occur 4-28 days after initial exposure to the drug, though case reports for delayed-onset (6 months) SJS/TEN to lamotrigine and rapid-onset SJS/TEN to acetaminophen and penicillin (3 days) have been reported. […] Because of the importance of MHC to the pathogenesis of SJS/TEN and other drug hypersensitivity reactions, many of the genetic variants most closely linked to the condition are HLA alleles. […] In particular, HLA-B*15:02 is strongly associated with aromatic anticonvulsant-associated SJS/TEN, with reported odds ratios (OR) for carbamazepine ranging from 17 to 1357 in white and Asian populations. […] A few other genetic risk factors shed light on the pathogenesis of SJS/TEN polymorphisms in cytochrome P450 and other genes required for drug metabolism, and polymorphisms in the antigen presentation pathway. […] Therefore, further research is needed to better understand these risk factors and effectively use them in a clinical setting.

• #102

  https://link.springer.com/article/10.1007/s40257-024-00889-6

  However, because SJS shares International Classification of Diseases, Tenth Revision (ICD-10) codes with erythema multiforme, incidence may be overestimated. […] Furthermore, 36-72% of patients initially diagnosed with SJS/TEN end up having their diagnosis reclassified into a different disease, also contributing to an overestimation of SJS/TEN incidence. […] The current understanding of SJS/TEN pathogenesis is summarized in Fig. 1. The key event is a tripartite interaction between a peptide presented by a major histocompatibility complex (MHC) on an antigen-presenting cell (APC) and a T cell receptor (TCR) expressed on a CD8+ (cytotoxic) T cell. […] Over 80% of cases are associated with medication exposure, particularly antimicrobials (sulfa antibiotics), antiepileptics, allopurinol, and nonsteroidal anti-inflammatory drugs (NSAIDs).

• #103 Toxic epidermal necrolysis and Stevens-Johnson syndrome | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-5-39

  Drug exposure and a resulting hypersensitivity reaction is the cause of the very large majority of cases of SJS/TEN. In absolute case numbers, allopurinol is the most common cause of SJS/TEN in Europe and Israel. […] The pathogenesis of SJS/TEN is not fully understood but is believed to be immune-mediated, as re-challenging an individual with the same drug can result in rapid recurrence of SJS/TEN. […] The histopathology of SJS/TEN lesions show that keratinocyte apoptosis followed by necrosis is the pathogenic basis of the widespread epidermal detachment observed in SJS/TEN.

• #104

  https://link.springer.com/article/10.1007/s40257-024-00889-6

  However, because SJS shares International Classification of Diseases, Tenth Revision (ICD-10) codes with erythema multiforme, incidence may be overestimated. […] Furthermore, 36-72% of patients initially diagnosed with SJS/TEN end up having their diagnosis reclassified into a different disease, also contributing to an overestimation of SJS/TEN incidence. […] The current understanding of SJS/TEN pathogenesis is summarized in Fig. 1. The key event is a tripartite interaction between a peptide presented by a major histocompatibility complex (MHC) on an antigen-presenting cell (APC) and a T cell receptor (TCR) expressed on a CD8+ (cytotoxic) T cell. […] Over 80% of cases are associated with medication exposure, particularly antimicrobials (sulfa antibiotics), antiepileptics, allopurinol, and nonsteroidal anti-inflammatory drugs (NSAIDs).

• #105 Stevens-Johnson Syndrome: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1197450-overview

  Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are characterized by significant keratinocyte apoptosis, epidermal necrosis, and separation between the dermis and epidermis. The apoptosis of keratinocytes is primarily driven by CD8 cytotoxic T cells and natural killer cells, which interact with human leukocyte antigens (HLA) and drug antigens. Genetic predispositions, particularly the strong association of HLA-B*15:02 with carbamazepine-induced SJS/TEN, have been identified in certain Asian populations, whereas no similar associations have been found in Japanese, Korean, or European populations. […] Various etiologic factors have been implicated as causes of Stevens-Johnson syndrome. Drugs most commonly are blamed. The 4 etiologic categories are as follows: Infectious, Drug-induced, Malignancy-related, Idiopathic. Stevens-Johnson syndrome is idiopathic in 25-50% of cases. Drugs and malignancies are most often implicated as the etiology in adults and elderly persons. Pediatric cases are related more often to infections.

• #106 Toxic epidermal necrolysis and Stevens-Johnson syndrome | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-5-39

  Toxic epidermal necrolysis (TEN) and Stevens Johnson Syndrome (SJS) are severe adverse cutaneous drug reactions that predominantly involve the skin and mucous membranes. […] Drugs are assumed or identified as the main cause of SJS/TEN in most cases, but Mycoplasma pneumoniae and Herpes simplex virus infections are well documented causes alongside rare cases in which the aetiology remains unknown. […] Genetic susceptibility to SJS and TEN is likely as exemplified by the strong association observed in Han Chinese between a genetic marker, the human leukocyte antigen HLA-B*1502, and SJS induced by carbamazepine. […] A second strong association between HLA genotype and SJS/TEN has been reported for allopurinol. Indeed, 100% of Han Chinese patients with a severe adverse drug reaction to allopurinol were HLA-B*5801 positive.

• #107 Stevens-Johnson Syndrome: Causes, Diagnosis, and Treatment

  https://www.healthline.com/health/stevens-johnson-syndrome

  Stevens-Johnson syndrome (SJS) is a rare and extremely serious condition that causes your skin to come loose and detach. […] In more than 80 percent of cases, SJS is caused by an unpredictable adverse reaction to medication. Its less frequently caused by infections. […] SJS is most commonly caused by an immune response to certain medications. Its also possible for some infections to cause SJS. […] Some experts have suggested that a combination of medications and infections could together cause SJS, while in some cases, the trigger is never identified. […] While more than 100 medications have been associated with SJS, they generally fall into a handful of categories. SJS develops within a few days to 8 weeks after first taking the new medication. […] There are two main categories of infections that are associated with SJS: viral and bacterial.

• #108 Stevens-Johnson Syndrome – EyeWiki

  https://eyewiki.org/Stevens-Johnson_Syndrome

  Stevens-Johnson syndrome (SJS) is a dermatologic emergency, characterized by the presence of epidermal and mucosal bullous lesions involving less than 10% of the total body surface area (TBSA). […] Up to 75% of cases are attributed to delayed drug hypersensitivity reactions to a medication or medication metabolite. […] The remaining 25% of SJS cases not attributable to medication hypersensitivity are believed to be caused by an infectious source. […] The vast majority of these cases are due to Mycoplasma pneumoniae infection. […] The mechanism for M. pneumoniae-induced SJS remains unclear. […] It has been discovered that specific HLA subtypes carry an increased risk for development of SJS in various populations after exposure to certain classes of medications. […] The incidence of SJS was found to be 100 times higher in individuals infected with HIV relative to the general population. […] Rapid introduction of high dosages of medications associated with SJS further increases a patients risk of developing SJS.

• #109 Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) – Skin Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/skin-disorders/hypersensitivity-and-reactive-skin-disorders/stevens-johnson-syndrome-sjs-and-toxic-epidermal-necrolysis-ten

  Stevens-Johnson syndrome and toxic epidermal necrolysis are commonly caused by medications or infections. […] Most cases of Stevens-Johnson syndrome and toxic epidermal necrolysis are caused by a reaction to a medication, most often sulfa and other antibiotics; antiseizure medications, such as phenytoin and carbamazepine; and certain other medications, such as piroxicam or allopurinol. […] Some cases are caused by a bacterial infection, vaccination, or graft-versus-host disease. In children with Stevens-Johnson syndrome, an infection is the most likely cause. […] These disorders occur in all age groups. They are more likely to occur in people with an abnormal immune system, such as those with a bone marrow transplant, in people with systemic lupus erythematosus, in people with other chronic joint and connective tissue diseases, or in people with human immunodeficiency virus (HIV) infection (particularly when people also have pneumonia caused by Pneumocystis jirovecii). The tendency to develop one of these disorders can run in families.

• #110

  https://link.springer.com/article/10.1007/s40257-024-00889-6

  More than 300 different drugs and supplements have been implicated. […] Most cases occur 4-28 days after initial exposure to the drug, though case reports for delayed-onset (6 months) SJS/TEN to lamotrigine and rapid-onset SJS/TEN to acetaminophen and penicillin (3 days) have been reported. […] Because of the importance of MHC to the pathogenesis of SJS/TEN and other drug hypersensitivity reactions, many of the genetic variants most closely linked to the condition are HLA alleles. […] In particular, HLA-B*15:02 is strongly associated with aromatic anticonvulsant-associated SJS/TEN, with reported odds ratios (OR) for carbamazepine ranging from 17 to 1357 in white and Asian populations. […] A few other genetic risk factors shed light on the pathogenesis of SJS/TEN polymorphisms in cytochrome P450 and other genes required for drug metabolism, and polymorphisms in the antigen presentation pathway. […] Therefore, further research is needed to better understand these risk factors and effectively use them in a clinical setting.

• #111 Toxic epidermal necrolysis and Stevens-Johnson syndrome | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-5-39

  Toxic epidermal necrolysis (TEN) and Stevens Johnson Syndrome (SJS) are severe adverse cutaneous drug reactions that predominantly involve the skin and mucous membranes. […] Drugs are assumed or identified as the main cause of SJS/TEN in most cases, but Mycoplasma pneumoniae and Herpes simplex virus infections are well documented causes alongside rare cases in which the aetiology remains unknown. […] Genetic susceptibility to SJS and TEN is likely as exemplified by the strong association observed in Han Chinese between a genetic marker, the human leukocyte antigen HLA-B*1502, and SJS induced by carbamazepine. […] A second strong association between HLA genotype and SJS/TEN has been reported for allopurinol. Indeed, 100% of Han Chinese patients with a severe adverse drug reaction to allopurinol were HLA-B*5801 positive.

• #112 Stevens-Johnson Syndrome – EyeWiki

  https://eyewiki.org/Stevens-Johnson_Syndrome

  Stevens-Johnson syndrome (SJS) is a dermatologic emergency, characterized by the presence of epidermal and mucosal bullous lesions involving less than 10% of the total body surface area (TBSA). […] Up to 75% of cases are attributed to delayed drug hypersensitivity reactions to a medication or medication metabolite. […] The remaining 25% of SJS cases not attributable to medication hypersensitivity are believed to be caused by an infectious source. […] The vast majority of these cases are due to Mycoplasma pneumoniae infection. […] The mechanism for M. pneumoniae-induced SJS remains unclear. […] It has been discovered that specific HLA subtypes carry an increased risk for development of SJS in various populations after exposure to certain classes of medications. […] The incidence of SJS was found to be 100 times higher in individuals infected with HIV relative to the general population. […] Rapid introduction of high dosages of medications associated with SJS further increases a patients risk of developing SJS.

• #113 Stevens-Johnson Syndrome (SJS): Causes, Rash & Treatments

  https://my.clevelandclinic.org/health/diseases/17656-stevens-johnson-syndrome

  Medications most likely to cause Stevens-Johnson syndrome include: Antibacterial sulfa drugs. […] You are at greater risk of SJS if you have the following conditions: Bone marrow transplant. […] SJS may redevelop if you are exposed to the same medication known to have triggered the condition the first time. In such cases, the second episode is usually more severe than the first episode.

• #114 Triggers for Stevens–Johnson syndrome / toxic epidermal necrolysis

  https://dermnetnz.org/topics/sjsten-triggers

  Medicines (listed alphabetically) Other causes […] This list of drugs known to cause SJS/TEN is not exclusive. […] Other causes […] Mycoplasma pneumoniae and cytomegalovirus infections […] Vaccinations […] Cancer, especially haematological cancers […] Systemic diseases […] Contrast medium […] External chemical exposure […] Herbal medicines […] Foods […] Bone marrow transplantation […] Radiotherapy.

• #115 Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) – Dermatologic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/dermatologic-disorders/hypersensitivity-and-reactive-skin-disorders/stevens-johnson-syndrome-sjs-and-toxic-epidermal-necrolysis-ten

  Stevens-Johnson syndrome and toxic epidermal necrolysis are severe cutaneous hypersensitivity reactions. Medications, especially sulfa drugs, antiseizure medications, and antibiotics, are the most common causes. […] Medications precipitate most cases of SJS and TEN. The most common medication causes include sulfa drugs (eg, sulfasalazine), other antibiotics (eg, aminopenicillins [usually ampicillin or amoxicillin], fluoroquinolones, cephalosporins), antiseizure medications (eg, phenytoin, carbamazepine, phenobarbital, valproic acid and its derivatives, lamotrigine), nonsteroidal anti-inflammatory drugs (eg, piroxicam, meloxicam), antiretroviral medications (eg, nevirapine), miscellaneous individual medications (eg, allopurinol, chlormezanone), and immune checkpoint inhibitors. […] Cases that are not caused by medications are attributed to infection (mostly with Mycoplasma pneumoniae), vaccination, and graft-vs-host disease. […] In children with Stevens-Johnson syndrome, an infection is the most likely cause, and most of those are related to M. pneumoniae.

• #116 Stevens-Johnson Syndrome: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1197450-overview

  Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are characterized by significant keratinocyte apoptosis, epidermal necrosis, and separation between the dermis and epidermis. The apoptosis of keratinocytes is primarily driven by CD8 cytotoxic T cells and natural killer cells, which interact with human leukocyte antigens (HLA) and drug antigens. Genetic predispositions, particularly the strong association of HLA-B*15:02 with carbamazepine-induced SJS/TEN, have been identified in certain Asian populations, whereas no similar associations have been found in Japanese, Korean, or European populations. […] Various etiologic factors have been implicated as causes of Stevens-Johnson syndrome. Drugs most commonly are blamed. The 4 etiologic categories are as follows: Infectious, Drug-induced, Malignancy-related, Idiopathic. Stevens-Johnson syndrome is idiopathic in 25-50% of cases. Drugs and malignancies are most often implicated as the etiology in adults and elderly persons. Pediatric cases are related more often to infections.

• #117 Stevens–Johnson syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Stevens%E2%80%93Johnson_syndrome

  Stevens-Johnson syndrome (SJS) is a type of severe skin reaction. […] The most common cause is certain medications such as lamotrigine, carbamazepine, allopurinol, sulfonamide antibiotics and nevirapine. […] Other causes can include infections such as Mycoplasma pneumoniae and cytomegalovirus, or the cause may remain unknown. […] SJS is thought to arise from a disorder of the immune system. […] The immune reaction can be triggered by drugs or infections. […] Genetic factors are associated with a predisposition to SJS. […] The cause of SJS is unknown in one-quarter to one-half of cases. […] SJS, SJS/TEN, and TEN are considered a single disease with common causes and mechanisms. […] Although SJS can be caused by viral infections and malignancies, the main cause is medications.

• #118

  https://link.springer.com/article/10.1007/s40257-024-00889-6

  However, because SJS shares International Classification of Diseases, Tenth Revision (ICD-10) codes with erythema multiforme, incidence may be overestimated. […] Furthermore, 36-72% of patients initially diagnosed with SJS/TEN end up having their diagnosis reclassified into a different disease, also contributing to an overestimation of SJS/TEN incidence. […] The current understanding of SJS/TEN pathogenesis is summarized in Fig. 1. The key event is a tripartite interaction between a peptide presented by a major histocompatibility complex (MHC) on an antigen-presenting cell (APC) and a T cell receptor (TCR) expressed on a CD8+ (cytotoxic) T cell. […] Over 80% of cases are associated with medication exposure, particularly antimicrobials (sulfa antibiotics), antiepileptics, allopurinol, and nonsteroidal anti-inflammatory drugs (NSAIDs).

• #119 Guidelines for the management of Stevens–Johnson syndrome/toxic epidermal necrolysis: An Indian perspective – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/guidelines-for-the-management-of-stevens-johnson-syndrome-toxic-epidermal-necrolysis-an-indian-perspective/

  The management essentials include early recognition of the condition, cessation of suspected drug(s) if any, prompt institution of supportive therapy, referral if required, initiation of specific therapy, management of complications and prevention of future episodes. […] The ideal therapy still remains a matter of debate as there are only a limited number of studies of good quality comparing the usefulness of different specific treatments. […] Disease-modifying therapy in SJS/TEN is aimed at halting the immunological processes leading to keratinocyte apoptosis. Various immunomodulating agents such as systemic corticosteroids, cyclosporine, intravenous immunoglobulin, plasmapheresis, tumor necrosis factor- inhibitors, granulocyte colony-stimulating factor and N-acetylcysteine have been used with variable results across the globe.

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