Formerly known as Lyell sickness, SJS (Stevens-Johnson syndrome), TEN (toxic epidermal necrolysis), and erythema multiform primary staphylococcal scalded epidermal syndrome are different manifestations of the same illness.
SJS, also referred to as toxic epidermal necrolysis, is a severe, rare, dangerous, & potentially lethal skin response that is characterized by systemic signs along with sheet-like dermal & mucosal breakdown.
In more than 80 percent of cases, medications constitute the root cause. The size of the detached surface of the skin area is used to categorize SJS/TEN.
Stevens-Johnson syndrome: body surface area significantly lower than 10 percent
10 percent to 30 percent of exposed surface overlap between Stevens-Johnson syndrome and toxic epidermal necrolysis
More than 30 percent of the surface of the skin is affected by toxic epidermal necrolysis
Epidemiology
It is estimated that 2 to 7 persons per million experience Stevens-Johnson syndrome/toxic epidermal necrolysis each year. 3 times more people experience Stevens-Johnson syndrome than toxic epidermal necrolysis.
Anyone with a genetic disposition can develop SJS/TEN, albeit it tends to strike older persons and women more frequently. HIV (Human immunodeficiency virus) infection is far more likely to cause it, with an estimated prevalence of 1/1000.
There have been many reports of medicines causing Stevens-Johnson syndrome or toxic epidermal necrolysis. Rarely are vaccinations and illnesses, including mycoplasma, CMV, & dengue, related to SJS/TEN.
The following medications are the most frequently linked to SJS/TEN:
Allopurinol: especially with daily doses of greater than 100 mg
Antibiotics: cephalosporins, penicillin, quinolones, and minocycline
When exposed to aromatic anticonvulsants & allopurinol, genetic variables such as HLA (human leukocyte antigen) allotypes enhance the risk of SJS/TEN.
SJS/TEN patients’ members of the family should be informed that they run the risk of contracting the condition & should exercise caution while using any medications linked to it.
The risk of SJS/TEN has been discovered so far in:
Han Chinese who take allopurinol and have the HLA-B*5801 mutation
Europeans who use abacavir or carbamazepine and have the HLA-B*5701 or HLA-A*3101 gene mutations.
Those who use aromatic anticonvulsants are Thai, Han Chinese, Malaysian, or South Indian.
Anatomy
Pathophysiology
The interaction or binding of a drug-binding metabolite or antigen with the MHC (major histocompatibility complex) type 1 and intracellular peptide to generate an immunogenic complex may be the first stage of SJS/TEN. It’s still being determined what the specific mechanism is. T-cells play a role in SJS/TEN.
Blister fluid contains CD8+ lymphocytes, which can cause keratinocyte death.
The immune defense system’s other cells also participate
Additionally present are CD40 ligand cells that can trigger the release of nitrous oxide, TNF-alpha, antibodies, and IL-8 (interleukin) that bind to cells. Apoptosis is also induced by TNF-alpha.
The cytokines Th1 & Th2 are both present.
Natural killer cells, Macrophages, and neutrophils are other cells connected to SJS/TEN. Drugs may bind to MHC-1 and the T cell receptors as a result of their pharmacologic involvement with the immune function. A pro-hapten theory is an alternate one, according to which drug compounds develop immune-stimulating properties.
Etiology
The Fas-Fas ligand (FasL) path that leads to apoptosis, granule-related exocytosis, death receptor/ TNF-alpha path, and drug-specific CD8+ cytolytic lymphocytes are all involved in the uncommon and unpredictable SJS/TEN reaction to the medication.
There are several mechanisms that are covered by current hypotheses.
Keratinocyte cell apoptosis is primarily brought on by granulysin, which is present in the cytotoxic granule.
Perforin and granzyme B are exocytosed by cytotoxic T lymphocytes, and as a result, channels are formed in the targeted cell surface, activating the caspases.
Fas-FasL, which is expressed on active cytotoxic T lymphocytes, can also kill keratinocytes by causing intrinsic caspases to be released.
Apoptosis may be induced by TNF-alpha or prevented by it.
Caspases may be stimulated by nitrous oxide generated by TNF-alpha & interferon-alpha.
Genetics
Prognostic Factors
The magnitude and severity upon presentation of SJS/TEN indicate that it may be quite dangerous and have a high death rate. The countrywide Inpatient Survey 2009 to 2012 (USA) found median corrected mortality rates of 14.8 percent for TEN, 4.8 percent for SJS, & 19.4 percent for SJS/TEN overlay.
SJS/TEN was detected in 66 out of 361 patients in Créteil, France. Of these, eighteen percent died; two percent had SJS, twelve percent had SJS/TEN overlay, & twenty-six percent had TEN. As a result of greater supportive therapy than in previous decades, there is a recent trend toward better mortality.
Clinical History
Physical Examination
Age group
Associated comorbidity
Associated activity
Acuity of presentation
Differential Diagnoses
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
by Stage
by Modality
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Future Trends
Media Gallary
References
https://www.ncbi.nlm.nih.gov/books/NBK459323/
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Formerly known as Lyell sickness, SJS (Stevens-Johnson syndrome), TEN (toxic epidermal necrolysis), and erythema multiform primary staphylococcal scalded epidermal syndrome are different manifestations of the same illness.
SJS, also referred to as toxic epidermal necrolysis, is a severe, rare, dangerous, & potentially lethal skin response that is characterized by systemic signs along with sheet-like dermal & mucosal breakdown.
In more than 80 percent of cases, medications constitute the root cause. The size of the detached surface of the skin area is used to categorize SJS/TEN.
Stevens-Johnson syndrome: body surface area significantly lower than 10 percent
10 percent to 30 percent of exposed surface overlap between Stevens-Johnson syndrome and toxic epidermal necrolysis
More than 30 percent of the surface of the skin is affected by toxic epidermal necrolysis
It is estimated that 2 to 7 persons per million experience Stevens-Johnson syndrome/toxic epidermal necrolysis each year. 3 times more people experience Stevens-Johnson syndrome than toxic epidermal necrolysis.
Anyone with a genetic disposition can develop SJS/TEN, albeit it tends to strike older persons and women more frequently. HIV (Human immunodeficiency virus) infection is far more likely to cause it, with an estimated prevalence of 1/1000.
There have been many reports of medicines causing Stevens-Johnson syndrome or toxic epidermal necrolysis. Rarely are vaccinations and illnesses, including mycoplasma, CMV, & dengue, related to SJS/TEN.
The following medications are the most frequently linked to SJS/TEN:
Allopurinol: especially with daily doses of greater than 100 mg
Antibiotics: cephalosporins, penicillin, quinolones, and minocycline
When exposed to aromatic anticonvulsants & allopurinol, genetic variables such as HLA (human leukocyte antigen) allotypes enhance the risk of SJS/TEN.
SJS/TEN patients’ members of the family should be informed that they run the risk of contracting the condition & should exercise caution while using any medications linked to it.
The risk of SJS/TEN has been discovered so far in:
Han Chinese who take allopurinol and have the HLA-B*5801 mutation
Europeans who use abacavir or carbamazepine and have the HLA-B*5701 or HLA-A*3101 gene mutations.
Those who use aromatic anticonvulsants are Thai, Han Chinese, Malaysian, or South Indian.
The interaction or binding of a drug-binding metabolite or antigen with the MHC (major histocompatibility complex) type 1 and intracellular peptide to generate an immunogenic complex may be the first stage of SJS/TEN. It’s still being determined what the specific mechanism is. T-cells play a role in SJS/TEN.
Blister fluid contains CD8+ lymphocytes, which can cause keratinocyte death.
The immune defense system’s other cells also participate
Additionally present are CD40 ligand cells that can trigger the release of nitrous oxide, TNF-alpha, antibodies, and IL-8 (interleukin) that bind to cells. Apoptosis is also induced by TNF-alpha.
The cytokines Th1 & Th2 are both present.
Natural killer cells, Macrophages, and neutrophils are other cells connected to SJS/TEN. Drugs may bind to MHC-1 and the T cell receptors as a result of their pharmacologic involvement with the immune function. A pro-hapten theory is an alternate one, according to which drug compounds develop immune-stimulating properties.
The Fas-Fas ligand (FasL) path that leads to apoptosis, granule-related exocytosis, death receptor/ TNF-alpha path, and drug-specific CD8+ cytolytic lymphocytes are all involved in the uncommon and unpredictable SJS/TEN reaction to the medication.
There are several mechanisms that are covered by current hypotheses.
Keratinocyte cell apoptosis is primarily brought on by granulysin, which is present in the cytotoxic granule.
Perforin and granzyme B are exocytosed by cytotoxic T lymphocytes, and as a result, channels are formed in the targeted cell surface, activating the caspases.
Fas-FasL, which is expressed on active cytotoxic T lymphocytes, can also kill keratinocytes by causing intrinsic caspases to be released.
Apoptosis may be induced by TNF-alpha or prevented by it.
Caspases may be stimulated by nitrous oxide generated by TNF-alpha & interferon-alpha.
The magnitude and severity upon presentation of SJS/TEN indicate that it may be quite dangerous and have a high death rate. The countrywide Inpatient Survey 2009 to 2012 (USA) found median corrected mortality rates of 14.8 percent for TEN, 4.8 percent for SJS, & 19.4 percent for SJS/TEN overlay.
SJS/TEN was detected in 66 out of 361 patients in Créteil, France. Of these, eighteen percent died; two percent had SJS, twelve percent had SJS/TEN overlay, & twenty-six percent had TEN. As a result of greater supportive therapy than in previous decades, there is a recent trend toward better mortality.
https://www.ncbi.nlm.nih.gov/books/NBK459323/
medtigo
Stevens Johnson Syndrome
Updated :
September 5, 2023
Formerly known as Lyell sickness, SJS (Stevens-Johnson syndrome), TEN (toxic epidermal necrolysis), and erythema multiform primary staphylococcal scalded epidermal syndrome are different manifestations of the same illness.
SJS, also referred to as toxic epidermal necrolysis, is a severe, rare, dangerous, & potentially lethal skin response that is characterized by systemic signs along with sheet-like dermal & mucosal breakdown.
In more than 80 percent of cases, medications constitute the root cause. The size of the detached surface of the skin area is used to categorize SJS/TEN.
Stevens-Johnson syndrome: body surface area significantly lower than 10 percent
10 percent to 30 percent of exposed surface overlap between Stevens-Johnson syndrome and toxic epidermal necrolysis
More than 30 percent of the surface of the skin is affected by toxic epidermal necrolysis
It is estimated that 2 to 7 persons per million experience Stevens-Johnson syndrome/toxic epidermal necrolysis each year. 3 times more people experience Stevens-Johnson syndrome than toxic epidermal necrolysis.
Anyone with a genetic disposition can develop SJS/TEN, albeit it tends to strike older persons and women more frequently. HIV (Human immunodeficiency virus) infection is far more likely to cause it, with an estimated prevalence of 1/1000.
There have been many reports of medicines causing Stevens-Johnson syndrome or toxic epidermal necrolysis. Rarely are vaccinations and illnesses, including mycoplasma, CMV, & dengue, related to SJS/TEN.
The following medications are the most frequently linked to SJS/TEN:
Allopurinol: especially with daily doses of greater than 100 mg
Antibiotics: cephalosporins, penicillin, quinolones, and minocycline
When exposed to aromatic anticonvulsants & allopurinol, genetic variables such as HLA (human leukocyte antigen) allotypes enhance the risk of SJS/TEN.
SJS/TEN patients’ members of the family should be informed that they run the risk of contracting the condition & should exercise caution while using any medications linked to it.
The risk of SJS/TEN has been discovered so far in:
Han Chinese who take allopurinol and have the HLA-B*5801 mutation
Europeans who use abacavir or carbamazepine and have the HLA-B*5701 or HLA-A*3101 gene mutations.
Those who use aromatic anticonvulsants are Thai, Han Chinese, Malaysian, or South Indian.
The interaction or binding of a drug-binding metabolite or antigen with the MHC (major histocompatibility complex) type 1 and intracellular peptide to generate an immunogenic complex may be the first stage of SJS/TEN. It’s still being determined what the specific mechanism is. T-cells play a role in SJS/TEN.
Blister fluid contains CD8+ lymphocytes, which can cause keratinocyte death.
The immune defense system’s other cells also participate
Additionally present are CD40 ligand cells that can trigger the release of nitrous oxide, TNF-alpha, antibodies, and IL-8 (interleukin) that bind to cells. Apoptosis is also induced by TNF-alpha.
The cytokines Th1 & Th2 are both present.
Natural killer cells, Macrophages, and neutrophils are other cells connected to SJS/TEN. Drugs may bind to MHC-1 and the T cell receptors as a result of their pharmacologic involvement with the immune function. A pro-hapten theory is an alternate one, according to which drug compounds develop immune-stimulating properties.
The Fas-Fas ligand (FasL) path that leads to apoptosis, granule-related exocytosis, death receptor/ TNF-alpha path, and drug-specific CD8+ cytolytic lymphocytes are all involved in the uncommon and unpredictable SJS/TEN reaction to the medication.
There are several mechanisms that are covered by current hypotheses.
Keratinocyte cell apoptosis is primarily brought on by granulysin, which is present in the cytotoxic granule.
Perforin and granzyme B are exocytosed by cytotoxic T lymphocytes, and as a result, channels are formed in the targeted cell surface, activating the caspases.
Fas-FasL, which is expressed on active cytotoxic T lymphocytes, can also kill keratinocytes by causing intrinsic caspases to be released.
Apoptosis may be induced by TNF-alpha or prevented by it.
Caspases may be stimulated by nitrous oxide generated by TNF-alpha & interferon-alpha.
The magnitude and severity upon presentation of SJS/TEN indicate that it may be quite dangerous and have a high death rate. The countrywide Inpatient Survey 2009 to 2012 (USA) found median corrected mortality rates of 14.8 percent for TEN, 4.8 percent for SJS, & 19.4 percent for SJS/TEN overlay.
SJS/TEN was detected in 66 out of 361 patients in Créteil, France. Of these, eighteen percent died; two percent had SJS, twelve percent had SJS/TEN overlay, & twenty-six percent had TEN. As a result of greater supportive therapy than in previous decades, there is a recent trend toward better mortality.
https://www.ncbi.nlm.nih.gov/books/NBK459323/
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