MCID: STV007
MIFTS: 47

Stevens-Johnson Syndrome/toxic Epidermal Necrolysis

Categories: Rare diseases

Aliases & Classifications for Stevens-Johnson Syndrome/toxic Epidermal Necrolysis

MalaCards integrated aliases for Stevens-Johnson Syndrome/toxic Epidermal Necrolysis:

Name: Stevens-Johnson Syndrome/toxic Epidermal Necrolysis 52 25
Stevens-Johnson Syndrome Toxic Epidermal Necrolysis Spectrum 52 25
Drug-Induced Stevens Johnson Syndrome 52 25
Toxic Epidermal Necrolysis 52 25
Stevens-Johnson Syndrome 52 25
Susceptibility to Severe Cutaneous Adverse Reaction Ity to 52
Mycoplasma-Induced Stevens Johnson Syndrome 25
Lyell's Syndrome 25
Sjs/ten 52

Classifications:



Summaries for Stevens-Johnson Syndrome/toxic Epidermal Necrolysis

NIH Rare Diseases : 52 Stevens-Johnson syndrome /toxic epidermal necrolysis (SJS/TEN) is a very severe reaction, most commonly triggered by medications, that causes skin tissue to die (necrosis) and detach. The mucous membranes of the eyes, mouth, and/or genitals are also commonly affected. SJS and TEN previously were thought to be separate conditions, but they are now considered part of a disease spectrum. SJS is at the less severe end of the spectrum, and TEN is at the more severe end. It is considered SJS when skin detachment involves less than 10% of the body surface, and TEN when skin detachment involves more than 30% of the body surface. People with skin detachment involving 10-30% of the body surface are said to have "SJS/TEN overlap." All forms of SJS/TEN are a medical emergency that can be life-threatening. The first symptoms of SJS/TEN often include fever and flu-like symptoms (such as general ill feeling, body aches, and cough). Within about 1 to 3 days, a red or purplish rash forms, and then the skin begins to blister and peel, leading to "raw" areas of skin that are painful. This often starts on the face and then spreads to other parts of the body. The mucous membranes may also become involved during this time, which can lead to symptoms such as severe conjunctivitis (when the eyes are affected), trouble swallowing and breathing (when the mouth and airway are affected), and difficulty urinating and genital pain (when the genitals are affected). SJS/TEN often is triggered by certain medications including allopurinol , anti-epileptics , pain relievers, cancer therapies, or antibiotics (sometimes up to 2 weeks after stopping the medication). SJS/TEN can also be triggered by infections such as pneumonia, herpes virus , and hepatitis A. In many cases the cause cannot be identified. People that may be at increased risk to develop SJS/TEN include those with HIV, a weakened immune system , a personal or family history of the condition, and certain variations of a gene called HLA-B . There are no universal diagnostic criteria for SJS/TEN. Currently the diagnosis is based on the person's medical history and symptoms. People suspected of having SJS/TEN should be admitted to the hospital to confirm the diagnosis and assess severity. As mentioned earlier, whether a person is diagnosed specifically with SJS, TEN, or SJS/TEN overlap depends on the percentage of body surface area affected. Treatment needs should be assessed in the hospital to determine severity and where treatment should be provided (e.g. intensive care unit, burn unit, or dermatology unit). Treatment may involve stopping a triggering medication (for those suspected of having medication-induced SJS/TEN), standard therapies used for major burns, various eye treatments (for those with eye involvement), pain control, and preventing and treating infections. The overall mortality rate is about 25%, ranging from about 10% for SJS to over 30% for TEN. The most common causes of death include sepsis , acute respiratory distress syndrome , and multiple organ failure. Those that survive may experience recurrence (particularly if re-exposed to a trigger) and/or long-term complications involving the skin and affected mucous membranes.

MalaCards based summary : Stevens-Johnson Syndrome/toxic Epidermal Necrolysis, also known as stevens-johnson syndrome toxic epidermal necrolysis spectrum, is related to severe cutaneous adverse reaction and paraneoplastic pemphigus. An important gene associated with Stevens-Johnson Syndrome/toxic Epidermal Necrolysis is HLA-B (Major Histocompatibility Complex, Class I, B), and among its related pathways/superpathways are ERK Signaling and PEDF Induced Signaling. The drugs Bezafibrate and Clotrimazole have been mentioned in the context of this disorder. Affiliated tissues include skin, eye and t cells, and related phenotypes are Decreased shRNA abundance (Z-score < -2) and Decreased shRNA abundance (Z-score < -2)

Genetics Home Reference : 25 Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a severe skin reaction most often triggered by particular medications. Although Stevens-Johnson syndrome and toxic epidermal necrolysis were once thought to be separate conditions, they are now considered part of a continuum. Stevens-Johnson syndrome represents the less severe end of the disease spectrum, and toxic epidermal necrolysis represents the more severe end. SJS/TEN often begins with a fever and flu-like symptoms. Within a few days, the skin begins to blister and peel, forming very painful raw areas called erosions that resemble a severe hot-water burn. The skin erosions usually start on the face and chest before spreading to other parts of the body. In most affected individuals, the condition also damages the mucous membranes, including the lining of the mouth and the airways, which can cause trouble with swallowing and breathing. The painful blistering can also affect the urinary tract and genitals. SJS/TEN often affects the eyes as well, causing irritation and redness of the conjunctiva, which are the mucous membranes that protect the white part of the eye and line the eyelids, and damage to the clear front covering of the eye (the cornea). Severe damage to the skin and mucous membranes makes SJS/TEN a life-threatening disease. Because the skin normally acts as a protective barrier, extensive skin damage can lead to a dangerous loss of fluids and allow infections to develop. Serious complications can include pneumonia, overwhelming bacterial infections (sepsis), shock, multiple organ failure, and death. About 10 percent of people with Stevens-Johnson syndrome die from the disease, while the condition is fatal in up to 50 percent of those with toxic epidermal necrolysis. Among people who survive, long-term effects of SJS/TEN can include changes in skin coloring (pigmentation), dryness of the skin and mucous membranes (xerosis), excess sweating (hyperhidrosis), hair loss (alopecia), and abnormal growth or loss of the fingernails and toenails. Other long-term problems can include impaired taste, difficulty urinating, and genital abnormalities. A small percentage of affected individuals develop chronic dryness or inflammation of the eyes, which can lead to increased sensitivity to light (photophobia) and vision impairment.

Related Diseases for Stevens-Johnson Syndrome/toxic Epidermal Necrolysis

Diseases related to Stevens-Johnson Syndrome/toxic Epidermal Necrolysis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 710)
# Related Disease Score Top Affiliating Genes
1 severe cutaneous adverse reaction 32.2 PSORS1C1 IKZF1 HLA-C HLA-B HLA-A FAS
2 paraneoplastic pemphigus 31.2 HLA-C HLA-B HLA-A
3 cutaneous lupus erythematosus 30.8 IFNG CXCR3 CCL17
4 fixed drug eruption 30.7 IL2 IFNG
5 cytomegalovirus infection 30.6 HLA-B HLA-A FAS CCL5
6 bronchiolitis obliterans 30.5 IL13 IFNG CXCR3
7 erythema multiforme 30.5 IL5 IL2 IL13 IFNG FAS CXCR3
8 viral meningitis 30.4 IFNG CXCR3 CCL5
9 bullous pemphigoid 30.4 IL5 IL13 CCL5 CCL17
10 syphilis 30.4 IFNG HLA-C FAS
11 cytokine deficiency 30.3 IL5 IL13
12 red cell aplasia 30.2 IL2 HLA-B HLA-A
13 pars planitis 30.2 IL2 HLA-B HLA-A
14 pure red-cell aplasia 30.2 IL2 HLA-B HLA-A
15 severe acute respiratory syndrome 30.2 IL2 IFNG HLA-A CCL5
16 smallpox 30.1 IL2 IFNG CXCR3
17 adult t-cell leukemia 30.1 IL2 FAS CCL22 CCL17
18 cellulitis 30.0 IL5 IL2 IFNG
19 uveitis 30.0 IL2 IFNG HLA-B HLA-A CXCR3
20 pyelonephritis 30.0 IFNG HLA-A CCL5
21 interstitial lung disease 30.0 IL5 IL13 IFNG CCL5 CCL17
22 lichen planus 30.0 IL2 IFNG CXCR3 CCL5
23 common cold 30.0 IL5 IL13 CCL5
24 respiratory failure 29.9 IL5 IL13 CCL5 CCL17
25 osteomyelitis 29.9 IL13 IFNG CCL5
26 folliculitis 29.9 IL5 IFNG CCL5
27 conjunctivitis 29.8 IL5 IL2 IL13 IFNG HLA-B
28 ulcerative colitis 29.8 IL2 IL13 IFNG CCR3
29 herpes zoster 29.8 IL2 IFNG HLA-B HLA-A
30 lymphopenia 29.8 IL2 IKZF1 IFNG FAS CCL22
31 chlamydia 29.8 TLR9 IL5 IFNG
32 atopic keratoconjunctivitis 29.8 IL5 IL2 IFNG CCL22
33 esophagitis 29.8 IL5 IL13 CCL5
34 ige responsiveness, atopic 29.8 IL5 IL13 IFNG
35 graft-versus-host disease 29.7 IL2 IFNG HLA-C HLA-B HLA-A FAS
36 melanoma, uveal 29.7 IL2 HLA-B HLA-A CXCR4
37 brucellosis 29.7 IL2 IL13 IFNG
38 chickenpox 29.6 TLR9 IL2 IFNG
39 bronchiolitis 29.6 TLR9 IL5 IL13 IFNG CCL5
40 tonsillitis 29.6 TLR9 IL2 IFNG
41 toxoplasmosis 29.6 TLR9 IL5 IFNG
42 hypereosinophilic syndrome 29.6 IL5 IL2 IL13 IFNG CCR3 CCL5
43 parasitic helminthiasis infectious disease 29.5 IL5 IL2 IL13
44 mycobacterium tuberculosis 1 29.5 TLR9 IFNG CXCR4 CXCR3
45 peripheral t-cell lymphoma 29.5 IL2 CXCR4 CXCR3
46 aspergillosis 29.5 TLR9 IL5 IFNG CCL5 CCL17
47 bronchitis 29.5 IL5 IL13 EPHX1 CCR3 CCL5
48 keratoconjunctivitis 29.4 IL5 IL2 IL13 IFNG CCL5
49 allergic contact dermatitis 29.4 IL5 IFNG CXCR3 CCL22 CCL17
50 glioblastoma multiforme 29.4 IL2 IL13 IFNG HLA-B FAS CXCR4

Graphical network of the top 20 diseases related to Stevens-Johnson Syndrome/toxic Epidermal Necrolysis:



Diseases related to Stevens-Johnson Syndrome/toxic Epidermal Necrolysis

Symptoms & Phenotypes for Stevens-Johnson Syndrome/toxic Epidermal Necrolysis

GenomeRNAi Phenotypes related to Stevens-Johnson Syndrome/toxic Epidermal Necrolysis according to GeneCards Suite gene sharing:

26 (show all 11)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased shRNA abundance (Z-score < -2) GR00366-A-103 10.02 HLA-A HLA-B HLA-C
2 Decreased shRNA abundance (Z-score < -2) GR00366-A-110 10.02 HLA-A HLA-B HLA-C
3 Decreased shRNA abundance (Z-score < -2) GR00366-A-14 10.02 HLA-C
4 Decreased shRNA abundance (Z-score < -2) GR00366-A-156 10.02 HLA-A HLA-B HLA-C
5 Decreased shRNA abundance (Z-score < -2) GR00366-A-178 10.02 HLA-A HLA-B HLA-C
6 Decreased shRNA abundance (Z-score < -2) GR00366-A-19 10.02 HLA-A HLA-B HLA-C
7 Decreased shRNA abundance (Z-score < -2) GR00366-A-202 10.02 HLA-C
8 Decreased shRNA abundance (Z-score < -2) GR00366-A-31 10.02 HLA-A HLA-B HLA-C
9 Decreased shRNA abundance (Z-score < -2) GR00366-A-7 10.02 HLA-A HLA-B HLA-C
10 Decreased shRNA abundance (Z-score < -2) GR00366-A-91 10.02 HLA-C
11 Decreased shRNA abundance (Z-score < -2) GR00366-A-95 10.02 HLA-C

MGI Mouse Phenotypes related to Stevens-Johnson Syndrome/toxic Epidermal Necrolysis:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10.1 CCL17 CCL5 CCR3 CXCR3 CXCR4 F2RL2
2 immune system MP:0005387 10 CCL17 CCL22 CCL5 CCR3 CXCR3 CXCR4
3 digestive/alimentary MP:0005381 9.92 CXCR4 FAS IFNG IKZF1 IL13 IL2
4 neoplasm MP:0002006 9.56 CXCR3 CXCR4 EPHX1 FAS IFNG IKZF1
5 respiratory system MP:0005388 9.28 CCR3 CXCR3 CXCR4 FAS IFNG IKZF1

Drugs & Therapeutics for Stevens-Johnson Syndrome/toxic Epidermal Necrolysis

Drugs for Stevens-Johnson Syndrome/toxic Epidermal Necrolysis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 128)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Bezafibrate Approved, Investigational Phase 4 41859-67-0 39042
2
Clotrimazole Approved, Vet_approved Phase 4 23593-75-1 2812
3
Miconazole Approved, Investigational, Vet_approved Phase 4 22916-47-8 4189
4
Gabapentin Approved, Investigational Phase 4 60142-96-3 3446
5
Phenytoin Approved, Vet_approved Phase 4 57-41-0 1775
6
Sargramostim Approved, Investigational Phase 4 83869-56-1, 123774-72-1
7
Lidocaine Approved, Vet_approved Phase 4 137-58-6 3676
8
Sotalol Approved Phase 4 3930-20-9, 959-24-0 5253
9 Molgramostim Investigational Phase 4 99283-10-0
10 Immunologic Factors Phase 4
11 Anticonvulsants Phase 4
12 Hypolipidemic Agents Phase 4
13 Lipid Regulating Agents Phase 4
14 Antimetabolites Phase 4
15 Analgesics, Non-Narcotic Phase 4
16 Immunosuppressive Agents Phase 4
17 Analgesics Phase 4
18 Antifungal Agents Phase 4
19 Cyclosporins Phase 4
20 Calcineurin Inhibitors Phase 4
21 Pharmaceutical Solutions Phase 4
22 Ophthalmic Solutions Phase 4
23 Psychotropic Drugs Phase 4
24 Analgesics, Opioid Phase 4
25 Excitatory Amino Acid Antagonists Phase 4
26 Anti-Anxiety Agents Phase 4
27 Sodium Channel Blockers Phase 4
28 Anti-Arrhythmia Agents Phase 4
29 Diuretics, Potassium Sparing Phase 4
30 Adrenergic Agents Phase 4
31 Adrenergic Antagonists Phase 4
32 Sympatholytics Phase 4
33 Adrenergic beta-Antagonists Phase 4
34 Neurotransmitter Agents Phase 4
35
Pioglitazone Approved, Investigational Phase 3 111025-46-8 4829
36
Sirolimus Approved, Investigational Phase 3 53123-88-9 5284616 6436030 46835353
37
Lesinurad Approved, Investigational Phase 3 878672-00-5 53465279
38
Efavirenz Approved, Investigational Phase 3 154598-52-4 64139
39
Etravirine Approved Phase 3 269055-15-4 193962
40
Prednisolone phosphate Approved, Vet_approved Phase 2, Phase 3 302-25-0
41
Methylprednisolone Approved, Vet_approved Phase 2, Phase 3 83-43-2 6741
42
Methylprednisolone hemisuccinate Approved Phase 2, Phase 3 2921-57-5
43
tannic acid Approved Phase 2, Phase 3 1401-55-4
44 Prednisolone acetate Approved, Vet_approved Phase 2, Phase 3 52-21-1
45
Prednisolone Approved, Vet_approved Phase 2, Phase 3 50-24-8 5755
46
Benzocaine Approved, Investigational Phase 2, Phase 3 94-09-7, 1994-09-7 2337
47
Azithromycin Approved Phase 2, Phase 3 83905-01-5 447043 55185
48
Lopinavir Approved Phase 2, Phase 3 192725-17-0 92727
49
Hydrocortisone Approved, Vet_approved Phase 2, Phase 3 50-23-7 5754
50
Hydrocortisone acetate Approved, Vet_approved Phase 2, Phase 3 50-03-3

Interventional clinical trials:

(show top 50) (show all 60)
# Name Status NCT ID Phase Drugs
1 Early Effect Of Bezafibrate On Fibrinogen Levels, Inflammatory Response And Clinical Impact, In Patients With ST Elevation Acute Myocardial Infarction Completed NCT02291796 Phase 4 Bezafibrate
2 Efficacy of 0.05% Cyclosporin Eye Drop in Stevens Johnson Syndrome Patient With Chronic Dry Eye Completed NCT01488396 Phase 4 0.05%cyclosporin eye drop
3 A Prospective, Randomized, Placebo-controlled Trial of Pre-transplant and Prompt Post-transplant Treatment With Azithromycin to Improve Early Allograft Function and Outcome After Lung Transplantation Completed NCT01915082 Phase 4 Azithromycin;Ora-Plus
4 Pain Control in Pediatric Posterior Spine Fusion Patients: The Effect of Gabapentin on Post-operative Opioid Use and Patient Satisfaction Completed NCT01977937 Phase 4 Gabapentin 250mg/5mL NDC:59762-5025-01;Simple Syrup
5 Phenytoin as an Augmentation for SSRI Failures: A Controlled Study Completed NCT00146237 Phase 4 phenytoin
6 Evaluation of G-CSF as a Treatment of Toxic Epidermal Necrolysis Recruiting NCT02739295 Phase 4 recombinant granulocyte - colony stimulating factor;NaCl 0.9%
7 Transdermal Lidocaine Patch for Post-Cesarean Pain Control for Women With Obesity: a Single-blind Randomized Controlled Trial Active, not recruiting NCT03810235 Phase 4 Transdermal Lidocaine Patch;Transdermal Hydrocolloid Placebo Patch
8 A Prospective, Multi-Center, Randomized, Open Label Trial to Determine if a Common Atrial Fibrillation Risk Locus Modulates Differential Response to Antiarrhythmic Drugs Not yet recruiting NCT02347111 Phase 4 Flecainide;Sotalol
9 Prevention of Coronary Artery in STENT Restenosis With the Combined Use of Pioglitazone and Sirolimus-Eluting Coronary Stent Unknown status NCT00376870 Phase 3 Pioglitazone;Placebo
10 NPB-01(Intravenous Immunoglobulin) Therapy for Patients With Stevens-Johnson Syndrome/ Toxic Epidermal Necrolysis Unresponsive to Corticosteroids. Completed NCT01696500 Phase 3 Intravenous immunoglobulin
11 Efficacy of Cultivated Corneal Epithelial Stem Cell for Ocular Surface Reconstruction Completed NCT01237600 Phase 2, Phase 3
12 Autologous ex Vivo Conjunctival Epithelial Cell Expansion for Ocular Surface Completed NCT00346450 Phase 3
13 A Phase 3 Randomized, Double-Blind, Multicenter, Placebo- Controlled Study to Assess the Efficacy and Safety of Lesinurad Monotherapy Compared to Placebo in Subjects With Gout and an Intolerance or Contraindication to a Xanthine Oxidase Inhibitor Completed NCT01508702 Phase 3 lesinurad;Placebo
14 A Phase III, Double Blind, Mulit-centre, Randomised Placebo Controlled, Pilot Study to Assess the Feasibility of Switching Individuals Receiving Efavirez With Continuing Central Nervous System (CNS) Toxicity to TMC125. Completed NCT00792324 Phase 3 Etravirine;Efavirenz
15 Randomised Evaluation of COVID-19 Therapy Recruiting NCT04381936 Phase 2, Phase 3 Lopinavir-Ritonavir;Corticosteroid;Hydroxychloroquine;Azithromycin;Tocilizumab
16 Maraviroc to Augment Rehabilitation Outcomes After Stroke Recruiting NCT03172026 Phase 2, Phase 3 Maraviroc 300 mg;Placebo 300 mg
17 NATIENS: A Phase III Randomized Double-Blinded Placebo Controlled Study to Determine the Optimal Management and Mechanisms of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Not yet recruiting NCT02987257 Phase 3 Harmonized supportive care;Cyclosporine 5 mg/kg bid days 0-14;Etanercept 50 mg sc day 0 and day 3
18 Lamictal in the Treatment of Post-Herpetic Neuralgia-A Safety, Efficacy,Randomized, Double Blind, Placebo Controlled, Cross-Over Study Terminated NCT00295776 Phase 2, Phase 3 Lamictal in the treatment of Post-Herpetic Neuralgia
19 The Use of Riboflavin/Ultraviolet A Cross-linked Human Donor Corneas as Carriers for the Boston Keratoprosthesis Completed NCT01582880 Phase 1, Phase 2 Riboflavin
20 Evaluation the Effect of Amniotic Membrane Extract Eye Drop (AMEED) on Human Corneal Epithelium Healing (Phase I and II Clinical Trial) Completed NCT02746848 Phase 1, Phase 2
21 Comparison of Standard Versus Double Dose of Amoxicillin in the Treatment of Non-Severe Pneumonia in Children Aged 2-59 Months: A Multi-Centre Randomized Double Blind Controlled Trial in Pakistan Completed NCT00130013 Phase 1, Phase 2 Oral Amoxicillin
22 Palifermin Treatment of Toxic Epidermal Necrolysis Terminated NCT02037347 Phase 1, Phase 2 Palifermin
23 Topical Infliximab in Autoimmune Eyes With Keratoprosthesis Withdrawn NCT02126020 Phase 1, Phase 2 topical infliximab
24 Infliximab Therapy to Improve Retention of the Boston Keratoprosthesis in Patients After Stevens Johnson Syndrome/ Toxic Epidermal Necrolysis Withdrawn NCT01256489 Phase 1, Phase 2 Infliximab
25 Pilot Study Comparing Remicade (Infliximab) vs. Standard Care in the Treatment of Toxic Epidermal Necrolysis Withdrawn NCT00372723 Phase 2 Remicaide (infliximab)
26 A Randomized Placebo Controlled Split-body Double-blind Phase II Clinical Trial to Investigate the Safety and Efficacy of Clobetasol 0.05% Ointment for the Treatment of Toxic Epidermal Necrolysis (TEN) Withdrawn NCT02319616 Phase 1, Phase 2 Clobetasol 0.05% ointment;Placebo
27 Assessment of Fibrin Glue in Pterygium Surgery and Other Forms of Ocular Surface Reconstruction Completed NCT00344201 Phase 1
28 Topical Infliximab for the Treatment of Sterile Corneal Melt Recruiting NCT02987686 Phase 1 Topical Infliximab
29 Platelet Rich-plasma in Management of Chronic Multiple Oral Ulcers Not yet recruiting NCT03878771 Phase 1 Dermovate cream in Orabase
30 Serum Interleukin -21 Level in Patients With Severe Adverse Cutaneous Drug Reaction and Correlation With Disease Severity. Unknown status NCT03166241
31 Evaluating the Effect of Isotretinoin in Regulatory T-cell Function in Adverse Cutaneous Drug Eruptions (ACDEs): A Pilot Study Unknown status NCT02795143 Isotretinoin
32 Comparison of Corneal Epitheliotropic Factors in Autologous Serum Eye Drops Between Nonautoimmune Dry Eye and Stevens-Johnson Syndrome With Dry Eye Unknown status NCT01122303
33 A Prospective Study to Prove the Usefulness of HLA-B*5801 Screening Test for the Prevention of Allopurinol-induced Severe Cutaneous Adverse Reaction in Patient With Chronic Kidney Disease Unknown status NCT03046914
34 Drug Patch Tests, Enzyme-linked Immunosorbent Spot Assay (Elispot) and Lymphocyte Transformation Tests in Patients With Severe Cutaneous Adverse Reaction to Drugs (SCARs) Unknown status NCT03176342
35 The Use of Pentacam in Assessment of Corneal Changes After Pterygium Excision Unknown status NCT03304366
36 Comparison of the Blister Fluid Components of Second Degree Burned Patients With the Blister Fluid Components Following Intralesional Cryosurgery of Keloid Scars - A Feasibility Study. Unknown status NCT01627769
37 The Effect of Sitagliptin on Hypertension, Arterial Stiffness, Oxidative Stress and Inflammation Unknown status NCT00696982 sitagliptin;glibenclamide
38 Evaluation of TNF-α Blockade Effect in Patients With Severe Cutaneous Adverse Drug Reactions (SCAR) Completed NCT01276314 anti- TNF-a;Prednisolone
39 Salivary Gland and Labial Mucous Membrane Transplantation in the Treatment of Severe Symblepharon and Dry Eye in Patients With Stevens-Johnson Syndrome. Completed NCT01178242
40 Stevens-Johnson Syndrome Associated With Antimicrobial Completed NCT00844038
41 A Prospective Open-label, Multicenter Clinical Investigation to Assess the Safety and Performance of ARGOS-IO System in Patients Undergoing Implantation of a Boston Keratoprosthesis (BKPro) Completed NCT02945176
42 Using Cognitive Tests and Functional MRI to Investigate Long Term Cognitive Dysfunction Following a Critical Illness Due to a Major Burn Injury Completed NCT03470844
43 Evaluation of Immune Responses to Different Antigens in Non Infectious Ocular Inflammatory Diseases Completed NCT00357071
44 Bacteremia Antibiotic Length Actually Needed for Clinical Effectiveness: A Pilot RCT Completed NCT02261506
45 Safety and Effectiveness of a Laboratory Intervention to Effectively NOT Treat Asymptomatic Bacteriuria (SALIENT-A) Completed NCT03445312
46 Association of Cytokines With the Development of Complications in Burn and Toxic Epidermal Necrolysis (TENS) Patients Recruiting NCT04252651
47 HLA Screening in Reducing the Risk of Antiepileptic Drug-induced Cutaneous Adverse Reactions: a Multicenter Clinical Study Recruiting NCT03184597
48 Adverse Cutaneous Drug Reactions Collection of Clinical Data and Biological Samples Recruiting NCT03659227
49 The Multicenter Registry of Patients With Severe Cutaneous Adverse Reactions Among Tertiary Medical Institutes in Thailand Recruiting NCT02574988
50 An Exploratory Case-Control Study of Genetic and Clinical Factors for Serious Cutaneous Reactions Among Users of Eslicarbazepine Acetate Recruiting NCT02520557

Search NIH Clinical Center for Stevens-Johnson Syndrome/toxic Epidermal Necrolysis

Genetic Tests for Stevens-Johnson Syndrome/toxic Epidermal Necrolysis

Anatomical Context for Stevens-Johnson Syndrome/toxic Epidermal Necrolysis

MalaCards organs/tissues related to Stevens-Johnson Syndrome/toxic Epidermal Necrolysis:

40
Skin, Eye, T Cells, Testes, Liver, Lung, Kidney

Publications for Stevens-Johnson Syndrome/toxic Epidermal Necrolysis

Articles related to Stevens-Johnson Syndrome/toxic Epidermal Necrolysis:

(show top 50) (show all 3397)
# Title Authors PMID Year
1
U.K. guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in adults 2016. 52 61
27317286 2016
2
Altered levels of complement components associated with non-immediate drug hypersensitivity reactions. 61
31795786 2020
3
[European Guidelines (S1) on the use of high-dose intravenous immunoglobulin in dermatology]. 61
32435817 2020
4
Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Children With Non-Hodgkin Lymphoma. 61
32576784 2020
5
Long-term outcomes of amniotic membrane treatment in acute Stevens-Johnson syndrome/toxic epidermal necrolysis. 61
32200005 2020
6
Expression of prostaglandin E2 receptor 3 in the eyelid epidermis of patients with Stevens-Johnson syndrome/toxic epidermal necrolysis. 61
31000507 2020
7
Treatment for oral lesions in pediatric patients with Stevens-Johnson's syndrome: A case report and literature review. 61
31923328 2020
8
Toxic Epidermal Necrolysis and Steven-Johnson Syndrome: A Comprehensive Review. 61
32520664 2020
9
Acute pancreatic injuries: A complication of Stevens-Johnson syndrome/toxic epidermal necrolysis associated with cytotoxic immune cell activation. 61
32561372 2020
10
Society of Dermatology Hospitalists supportive care guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in adults. 61
32151629 2020
11
Differences in treatment of Stevens-Johnson syndrome and toxic epidermal necrolysis at burn centers and non-burn centers. 61
32498082 2020
12
[Advances in understanding of the pathophysiology of epidermal necrolysis (Stevens-Johnson syndrome and toxic epidermal necrolysis)]. 61
32253008 2020
13
Stevens-Johnson syndrome and toxic epidermal necrolysis-like reactions to checkpoint inhibitors: a systematic review. 61
32052409 2020
14
Long-term Progression of Ocular Surface Disease in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. 61
32028281 2020
15
A pediatric case of Stevens-Johnson syndrome/toxic epidermal necrolysis with rapid response to intravenous cyclosporine. 61
32509946 2020
16
Retrospective study of 213 cases of Stevens-Johnson syndrome and toxic epidermal necrolysis from China. 61
31898979 2020
17
Erythema multiforme, Stevens-Johnson syndrome/toxic epidermal necrolysis - diagnosis and treatment. 61
32469468 2020
18
Mycoplasma Pneumoniae Induced Rash and Mucositis (MIRM): A Longitudinal Perspective and Proposed Management Criteria. 61
32574770 2020
19
The Use of TNFα Inhibitors in Treating Pediatric Skin Disorders. 61
32350817 2020
20
Fixed Drug Eruptions: An Update, Emphasizing the Potentially Lethal Generalized Bullous Fixed Drug Eruption. 61
32002848 2020
21
An update on CYP2C9 polymorphisms and phenytoin metabolism: implications for adverse effects. 61
32510242 2020
22
Association of carbamazepine-induced Stevens-Johnson syndrome/toxic epidermal necrolysis with the HLA-B75 serotype or HLA-B*15:21 allele in Filipino patients. 61
31896765 2020
23
Clinical trial to evaluate the therapeutic benefits of limbal-supported contact lens wear for ocular sequelae due to Stevens-Johnson syndrome/toxic epidermal necrolysis. 61
32513522 2020
24
Reply to: "The age of procalcitonin: Potential pitfalls in critically ill patients with SJS/TEN. A comment on 'Risk factors and diagnostic markers of bacteremia in Stevens-Johnson syndrome and toxic epidermal necrolysis: A cohort study of 176 patients'". 61
32092373 2020
25
The age of procalcitonin: potential pitfalls in critically ill patients with SJS/TEN. A comment on "Risk factors and diagnostic markers of bacteremia in Stevens-Johnson syndrome and toxic epidermal necrolysis: A cohort study of 176 patients". 61
32092376 2020
26
Burn center admissions of patients with autoimmune rheumatic diseases: clinical characteristics and outcomes. 61
32507914 2020
27
General characteristics, economic burden, causative drugs and medical errors associated with medical damage litigation involving severe cutaneous adverse drug reactions in China. 61
32516456 2020
28
Burns vs. toxic epidermal necrolysis: A medico-legal dilemma. 61
32502359 2020
29
Toxic epidermal necrolysis with severe hepatic involvement. 61
32391959 2020
30
Toxicological screening reveals toxic epidermal necrolysis likely carbamazepine-induced rather than idiopathic. 61
32004748 2020
31
Paraneoplastic Pemphigus Mimicking Toxic Epidermal Necrolysis Associated with Follicular Lymphoma: Possible Pathological Role of CD8 T Cells. 61
32516424 2020
32
The frequency of low-risk morbilliform drug eruptions observed in patients treated with different classes of antibiotics. 61
31681983 2020
33
Direct Injection of 5-Fluorouracil Improves Outcomes in Cicatrizing Conjunctival Disorders Secondary to Systemic Disease. 61
32427724 2020
34
A case of drug-induced bullous pemphigoid associated with teriflunomide: A patient with relapsing multiple sclerosis. 61
32446168 2020
35
A young male with epitheliolysis in the small bowel and colon. 61
32393134 2020
36
Ventilatory support in Stevens-Johnson syndrome and toxic epidermal necrolysis: a systematic review and meta-analysis. 61
32412819 2020
37
Upper airway involvement in Stevens-Johnson syndrome and toxic epidermal necrolysis. 61
31591001 2020
38
RIP3 as a diagnostic and severity marker for Stevens-Johnson syndrome and toxic epidermal necrolysis. 61
31954192 2020
39
The effects of systemic cyclosporine in acute Stevens-Johnson syndrome/toxic epidermal necrolysis on ocular disease. 61
32445836 2020
40
Erythema Multiforme Versus Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: Subtle Difference in Presentation, Major Difference in Management. 61
32373930 2020
41
Association of HLA genotypes with phenytoin induced severe cutaneous adverse drug reactions in Thai children. 61
32272329 2020
42
Two False Negative Test Results in a Symptomatic Patient with a Confirmed Case of Severe Acute Respiratory Syndrome Coronavirus-2 (SARS-CoV-2) and Suspected Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN). 61
32455090 2020
43
Cutaneous toxicities of new targeted cancer therapies: must know for diagnosis, management, and patient-proxy empowerment. 61
31594365 2020
44
Genetic association of co-trimoxazole-induced severe cutaneous adverse reactions is phenotype-specific: HLA class I genotypes and haplotypes. 61
32452529 2020
45
Toxic epidermal necrolysis syndrome induced by tigecycline: a case report. 61
32400243 2020
46
Genetic variants associated with T cell-mediated cutaneous adverse drug reactions: A PRISMA-compliant systematic review-An EAACI position paper. 61
31899808 2020
47
A case of zonisamide-induced toxic epidermal necrolysis with acute respiratory failure. 61
32439282 2020
48
Bullous pemphigoid secondary to pembrolizumab mimicking toxic epidermal necrolysis. 61
32382629 2020
49
HLA-B*58: 01 association in allopurinol-induced severe cutaneous adverse reactions: the implication of ethnicity and clinical phenotypes in multiethnic Malaysia. 61
32433341 2020
50
Acute generalized exanthematous pustulosis with features mimicking toxic epidermal necrolysis secondary to amiodarone. 61
32522449 2020

Variations for Stevens-Johnson Syndrome/toxic Epidermal Necrolysis

Expression for Stevens-Johnson Syndrome/toxic Epidermal Necrolysis

Search GEO for disease gene expression data for Stevens-Johnson Syndrome/toxic Epidermal Necrolysis.

Pathways for Stevens-Johnson Syndrome/toxic Epidermal Necrolysis

Pathways related to Stevens-Johnson Syndrome/toxic Epidermal Necrolysis according to GeneCards Suite gene sharing:

(show all 47)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.81 IL5 IL2 IL13 FAS CXCR4 CXCR3
2
Show member pathways
13.62 IL5 IL2 IL13 IFNG FAS CXCR4
3
Show member pathways
13.52 IL5 IL2 IL13 IFNG F2RL2 CXCR4
4
Show member pathways
13.32 IL5 IL2 IL13 IFNG HLA-C HLA-B
5
Show member pathways
13.21 TLR9 IL5 IL2 IL13 FAS CXCR4
6
Show member pathways
13.17 IL5 IL2 IL13 FAS CXCR4 CXCR3
7
Show member pathways
12.92 HLA-C HLA-B HLA-A FAS CXCR4 CCR3
8
Show member pathways
12.85 FAS CXCR4 CXCR3 CCR3 CCL5 CCL22
9 12.82 IL5 IL2 IL13 IFNG FAS CXCR4
10 12.75 TLR9 IFNG HLA-C HLA-B HLA-A FAS
11
Show member pathways
12.68 TLR9 IL2 IFNG HLA-C HLA-B HLA-A
12
Show member pathways
12.61 TLR9 IL5 IL2 IFNG FAS
13
Show member pathways
12.55 IL5 IL2 IL13 IFNG HLA-C HLA-B
14
Show member pathways
12.49 IFNG HLA-C HLA-B HLA-A
15 12.49 TLR9 IL2 IKZF1 IFNG FAS CXCR4
16
Show member pathways
12.48 IL2 IFNG HLA-C HLA-B HLA-A FAS
17
Show member pathways
12.46 IL5 IL2 IL13 IFNG CXCR4
18 12.39 HLA-C HLA-B HLA-A CXCR4
19 12.33 IL2 HLA-C HLA-B HLA-A
20
Show member pathways
12.32 IL5 IL13 IFNG CCL17
21 12.3 HLA-C HLA-B HLA-A CCR3
22
Show member pathways
12.21 TLR9 IL2 CCL22 CCL17
23
Show member pathways
12.21 IL5 IL2 IL13 IFNG HLA-C HLA-B
24
Show member pathways
12.14 IL2 CXCR4 CXCR3 CCR3 CCL5 CCL22
25
Show member pathways
12.04 TLR9 IL5 IL2 IL13
26 11.98 IL5 IL2 IFNG CCR3
27
Show member pathways
11.9 TLR9 IL2 IFNG
28 11.81 IL5 IL2 IL13 IFNG
29
Show member pathways
11.77 IL5 IL2 IKZF1 IFNG
30 11.75 TLR9 IL2 IKZF1 IFNG
31 11.67 IL5 IL13 CCR3 CCL5
32
Show member pathways
11.64 IL2 IFNG HLA-A
33 11.56 IL2 IFNG FAS
34 11.54 IL13 CCL5 CCL22
35 11.53 IL5 IKZF1 CXCR4
36 11.4 TLR9 IFNG FAS
37
Show member pathways
11.39 IL2 IFNG FAS
38 11.34 IL5 IL2 IFNG
39 11.34 IL5 IL2 IL13 IKZF1 IFNG CXCR3
40 11.31 IL5 IL13 IFNG
41 11.26 IL5 IL2 IL13 IFNG
42 11.23 IL2 IL13 IFNG
43 11.19 IL5 IL2 IL13 IFNG
44 11.18 HLA-C HLA-B HLA-A
45 11.03 IL5 IL2 IL13 CCL5 CCL22 CCL17
46 10.77 IL5 IL13
47 10.76 IL5 IL2 IL13 IFNG CCL22 CCL17

GO Terms for Stevens-Johnson Syndrome/toxic Epidermal Necrolysis

Cellular components related to Stevens-Johnson Syndrome/toxic Epidermal Necrolysis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cell GO:0005623 9.91 IL2 IL13 F2RL2 CXCR4 CXCR3 CCR3
2 extracellular region GO:0005576 9.85 TLR9 IL5 IL2 IL13 IFNG FAS
3 cell surface GO:0009986 9.73 HLA-C HLA-B HLA-A FAS CXCR4 CXCR3
4 external side of plasma membrane GO:0009897 9.72 IL13 FAS CXCR4 CXCR3 CCR3
5 recycling endosome membrane GO:0055038 9.63 HLA-C HLA-B HLA-A
6 phagocytic vesicle membrane GO:0030670 9.61 HLA-C HLA-B HLA-A
7 ER to Golgi transport vesicle membrane GO:0012507 9.58 HLA-C HLA-B HLA-A
8 integral component of lumenal side of endoplasmic reticulum membrane GO:0071556 9.13 HLA-C HLA-B HLA-A
9 MHC class I protein complex GO:0042612 8.8 HLA-C HLA-B HLA-A

Biological processes related to Stevens-Johnson Syndrome/toxic Epidermal Necrolysis according to GeneCards Suite gene sharing:

(show all 36)
# Name GO ID Score Top Affiliating Genes
1 signal transduction GO:0007165 10.23 TLR9 IL5 FAS F2RL2 CXCR4 CXCR3
2 G protein-coupled receptor signaling pathway GO:0007186 10.1 IL2 F2RL2 CXCR4 CXCR3 CCR3 CCL5
3 viral process GO:0016032 10.05 HLA-C HLA-B HLA-A CXCR4 CCR3
4 adaptive immune response GO:0002250 10.01 IL2 IFNG HLA-C HLA-B HLA-A
5 cell-cell signaling GO:0007267 9.93 IL2 CCL5 CCL22 CCL17
6 cytokine-mediated signaling pathway GO:0019221 9.93 IL5 IL2 IL13 CCL5 CCL22
7 positive regulation of cytosolic calcium ion concentration GO:0007204 9.87 IL2 CXCR4 CXCR3 CCR3
8 cellular response to interferon-gamma GO:0071346 9.83 CCL5 CCL22 CCL17
9 positive regulation of inflammatory response GO:0050729 9.83 TLR9 IL2 IFNG
10 calcium-mediated signaling GO:0019722 9.81 CXCR4 CXCR3 CCR3
11 cellular response to interleukin-1 GO:0071347 9.81 CCL5 CCL22 CCL17
12 response to virus GO:0009615 9.81 IFNG CXCR4 CCL5 CCL22
13 neutrophil chemotaxis GO:0030593 9.8 CCL5 CCL22 CCL17
14 antigen processing and presentation of exogenous peptide antigen via MHC class I, TAP-dependent GO:0002479 9.79 HLA-C HLA-B HLA-A
15 type I interferon signaling pathway GO:0060337 9.78 HLA-C HLA-B HLA-A
16 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.78 IL2 IL13 IFNG CCL5
17 monocyte chemotaxis GO:0002548 9.76 CCL5 CCL22 CCL17
18 interferon-gamma-mediated signaling pathway GO:0060333 9.76 IFNG HLA-C HLA-B HLA-A
19 antigen processing and presentation GO:0019882 9.73 HLA-C HLA-B HLA-A
20 lymphocyte chemotaxis GO:0048247 9.72 CCL5 CCL22 CCL17
21 cell chemotaxis GO:0060326 9.72 CXCR4 CXCR3 CCR3 CCL5 CCL22
22 antigen processing and presentation of peptide antigen via MHC class I GO:0002474 9.7 HLA-C HLA-B HLA-A
23 dendritic cell chemotaxis GO:0002407 9.67 CXCR4 CCL5
24 regulation of regulatory T cell differentiation GO:0045589 9.67 IL2 IFNG
25 positive regulation of B cell proliferation GO:0030890 9.67 TLR9 IL5 IL2 IL13
26 positive regulation of nitric-oxide synthase biosynthetic process GO:0051770 9.66 TLR9 IFNG
27 positive regulation of immunoglobulin secretion GO:0051024 9.65 IL5 IL2
28 chemokine-mediated signaling pathway GO:0070098 9.65 CXCR3 CCR3 CCL5 CCL22 CCL17
29 detection of bacterium GO:0016045 9.64 HLA-B HLA-A
30 chemotaxis GO:0006935 9.63 CXCR4 CXCR3 CCR3 CCL5 CCL22 CCL17
31 antigen processing and presentation of exogenous peptide antigen via MHC class I, TAP-independent GO:0002480 9.61 HLA-C HLA-B HLA-A
32 inflammatory response GO:0006954 9.61 TLR9 IL5 IL13 CXCR4 CXCR3 CCR3
33 positive regulation of immunoglobulin production GO:0002639 9.6 TLR9 IL13
34 protection from natural killer cell mediated cytotoxicity GO:0042270 9.59 HLA-B HLA-A
35 antigen processing and presentation of endogenous peptide antigen via MHC class I via ER pathway, TAP-independent GO:0002486 9.58 HLA-C HLA-B HLA-A
36 immune response GO:0006955 9.5 TLR9 IL5 IL2 IL13 IFNG HLA-C

Molecular functions related to Stevens-Johnson Syndrome/toxic Epidermal Necrolysis according to GeneCards Suite gene sharing:

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 chemokine activity GO:0008009 9.65 CCL5 CCL22 CCL17
2 peptide antigen binding GO:0042605 9.63 HLA-C HLA-B HLA-A
3 CCR chemokine receptor binding GO:0048020 9.61 CCL5 CCL22 CCL17
4 chemokine binding GO:0019956 9.54 CXCR4 CXCR3 CCR3
5 C-C chemokine binding GO:0019957 9.5 CXCR4 CXCR3 CCR3
6 C-X-C chemokine receptor activity GO:0016494 9.49 CXCR4 CXCR3
7 C-C chemokine receptor activity GO:0016493 9.43 CXCR4 CXCR3 CCR3
8 CCR4 chemokine receptor binding GO:0031729 9.4 CCL5 CCL17
9 chemokine receptor activity GO:0004950 9.33 CXCR4 CXCR3 CCR3
10 cytokine activity GO:0005125 9.17 IL5 IL2 IL13 IFNG CCL5 CCL22
11 TAP binding GO:0046977 9.13 HLA-C HLA-B HLA-A

Sources for Stevens-Johnson Syndrome/toxic Epidermal Necrolysis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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