MCID: STV007
MIFTS: 52

Stevens-Johnson Syndrome/toxic Epidermal Necrolysis

Categories: Rare diseases, Skin 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 54 26
Stevens-Johnson Syndrome Toxic Epidermal Necrolysis Spectrum 54 26
Drug-Induced Stevens Johnson Syndrome 54 26
Toxic Epidermal Necrolysis 54 26
Stevens-Johnson Syndrome 54 26
Susceptibility to Severe Cutaneous Adverse Reaction Ity to 54
Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis 38
Mycoplasma-Induced Stevens Johnson Syndrome 26
Lyell's Syndrome 26
Sjs/ten 54

Classifications:



External Ids:

KEGG 38 H01694

Summaries for Stevens-Johnson Syndrome/toxic Epidermal Necrolysis

NIH Rare Diseases : 54 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 erythema multiforme. 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 Miconazole and Sulfadoxine have been mentioned in the context of this disorder. Affiliated tissues include skin, eye and salivary gland, and related phenotypes are Increased shRNA abundance (Z-score > 2) and Increased shRNA abundance (Z-score > 2)

Genetics Home Reference : 26 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.

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 360)
# Related Disease Score Top Affiliating Genes
1 severe cutaneous adverse reaction 32.2 EPHX1 FAS HLA-A HLA-B HLA-C IKZF1
2 erythema multiforme 31.7 CCL17 CXCR3 IFNG IL2
3 fixed drug eruption 31.1 IFNG IL2
4 conjunctivitis 31.0 IFNG IL13 IL2 IL5
5 acquired immunodeficiency syndrome 30.8 HLA-B IFNG IL2
6 tetanus 30.7 IFNG IL13 IL2 IL5
7 cytokine deficiency 30.6 IL13 IL5
8 uveitis 30.6 CXCR3 HLA-B IFNG
9 folliculitis 30.6 CCL5 IFNG IL5
10 paraneoplastic pemphigus 30.5 HLA-A HLA-B HLA-C
11 contact dermatitis 30.5 CCL17 CCL22 CXCR3 IFNG IL5
12 immunodysregulation, polyendocrinopathy, and enteropathy, x-linked 30.5 CXCR4 IFNG IL2
13 atopic keratoconjunctivitis 30.5 CCL22 IFNG IL2 IL5
14 hypereosinophilic syndrome 30.5 CCL17 IL13 IL5
15 pneumonia 30.4 CCL5 IL13 IL5 TLR9
16 herpes zoster 30.4 HLA-A HLA-B IFNG IL2
17 influenza 30.3 CCL5 HLA-B IFNG TLR9
18 allergic contact dermatitis 30.2 CCL17 CXCR3 IFNG IL5
19 hematopoietic stem cell transplantation 30.2 HLA-A HLA-B HLA-C IFNG IL2
20 lymphopenia 30.2 FAS IFNG IL2
21 pulmonary tuberculosis 30.2 IFNG IL2 TLR9
22 graft-versus-host disease 30.1 FAS HLA-A HLA-B HLA-C IFNG IL2
23 visceral leishmaniasis 30.0 IFNG IL2 TLR9
24 leishmaniasis 30.0 IFNG IL13 IL5 TLR9
25 cytomegalovirus infection 30.0 CCL5 FAS HLA-A HLA-B
26 dermatitis 30.0 CCL17 CCL22 IFNG IL13 IL5 TLR9
27 myeloma, multiple 30.0 CXCR4 IKZF1 IL2 TLR9
28 yellow fever 29.9 CCL5 IL5 TLR9
29 lung disease 29.9 CCL5 CXCR3 IL13 IL5 TLR9
30 human immunodeficiency virus infectious disease 29.9 CCL5 CXCR4 IFNG IL2 TLR9
31 keratoconjunctivitis 29.8 CCL17 IFNG IL13 IL2 IL5 TLR9
32 lichen planus 29.8 CXCR3 IFNG TLR9
33 aplastic anemia 29.8 FAS HLA-A HLA-B IFNG IL2
34 aspergillosis 29.8 CCL17 IFNG IL5 TLR9
35 mycosis fungoides 29.8 CCL17 CCL22 CCR3 CXCR3 IL2 IL5
36 autoimmune disease 29.6 FAS IFNG IL2 TLR9
37 bronchiolitis 29.6 CCL5 IFNG IL13 IL5 TLR9
38 systemic lupus erythematosus 29.5 CCL5 CXCR3 FAS IFNG IL2 TLR9
39 lymphoma, non-hodgkin, familial 29.4 CXCR4 FAS IL2 TLR9
40 human immunodeficiency virus type 1 29.1 CCL5 CCR3 CXCR4 HLA-A HLA-B HLA-C
41 skin disease 29.1 CCL17 HLA-B HLA-C IFNG IL13 IL5
42 allergic hypersensitivity disease 29.0 CCL17 CCL22 CCL5 CCR3 IFNG IL13
43 malaria 28.8 CCL5 CXCR3 HLA-A HLA-B IFNG IL13
44 stevens-johnson syndrome/toxic epidermal necrolysis overlap syndrome 13.0
45 ritter's disease 11.8
46 x-linked intellectual disability-macrocephaly-macroorchidism syndrome 11.4
47 5-oxoprolinase deficiency 11.4
48 auditory neuropathy spectrum disorder 11.4
49 burns 10.5
50 lupus erythematosus 10.5

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:

27 (show all 15)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-103 10.14 HLA-A HLA-B HLA-C
2 Increased shRNA abundance (Z-score > 2) GR00366-A-110 10.14 HLA-A HLA-B HLA-C
3 Increased shRNA abundance (Z-score > 2) GR00366-A-118 10.14 HLA-A HLA-B HLA-C
4 Increased shRNA abundance (Z-score > 2) GR00366-A-124 10.14 HLA-C
5 Increased shRNA abundance (Z-score > 2) GR00366-A-149 10.14 HLA-C
6 Increased shRNA abundance (Z-score > 2) GR00366-A-156 10.14 HLA-A HLA-B HLA-C
7 Increased shRNA abundance (Z-score > 2) GR00366-A-162 10.14 HLA-C
8 Increased shRNA abundance (Z-score > 2) GR00366-A-178 10.14 HLA-A HLA-B HLA-C
9 Increased shRNA abundance (Z-score > 2) GR00366-A-19 10.14 HLA-A HLA-B HLA-C
10 Increased shRNA abundance (Z-score > 2) GR00366-A-202 10.14 HLA-C
11 Increased shRNA abundance (Z-score > 2) GR00366-A-31 10.14 HLA-A HLA-B HLA-C
12 Increased shRNA abundance (Z-score > 2) GR00366-A-47 10.14 HLA-C
13 Increased shRNA abundance (Z-score > 2) GR00366-A-7 10.14 HLA-A HLA-B HLA-C
14 Increased shRNA abundance (Z-score > 2) GR00366-A-74 10.14 HLA-A HLA-B HLA-C
15 Increased shRNA abundance (Z-score > 2) GR00366-A-93 10.14 HLA-C

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

47
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 10.07 CCL17 CCR3 CXCR3 CXCR4 FAS IFNG
2 hematopoietic system MP:0005397 10.07 CCL17 CCL5 CCR3 CXCR3 CXCR4 F2RL2
3 immune system MP:0005387 9.97 CCL17 CCL22 CCL5 CCR3 CXCR3 CXCR4
4 digestive/alimentary MP:0005381 9.92 CXCR4 FAS IFNG IKZF1 IL13 IL2
5 neoplasm MP:0002006 9.56 CXCR3 CXCR4 EPHX1 FAS IFNG IKZF1
6 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 151)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Miconazole Approved, Investigational, Vet_approved Phase 4,Phase 3 22916-47-8 4189
2
Sulfadoxine Approved, Investigational Phase 4 2447-57-6 17134
3
Pyrimethamine Approved, Investigational, Vet_approved Phase 4 58-14-0 4993
4
leucovorin Approved Phase 4 58-05-9 6006 143
5
Pantoprazole Approved Phase 4 102625-70-7 4679
6
Sargramostim Approved, Investigational Phase 4 123774-72-1, 83869-56-1
7
Folic Acid Approved, Nutraceutical, Vet_approved Phase 4,Phase 1,Phase 2 59-30-3 6037
8 Ophthalmic Solutions Phase 4,Phase 1,Phase 2
9 Antirheumatic Agents Phase 4,Phase 3,Phase 1,Phase 2,Not Applicable
10 Pharmaceutical Solutions Phase 4,Phase 3,Phase 1,Phase 2
11 Anti-Infective Agents Phase 4,Phase 3,Phase 2,Not Applicable
12 Immunosuppressive Agents Phase 4,Phase 3,Not Applicable
13 Immunologic Factors Phase 4,Phase 3,Not Applicable
14 Cyclosporins Phase 4,Phase 3
15 Antifungal Agents Phase 4,Phase 3
16 Dermatologic Agents Phase 4,Phase 3,Phase 1,Phase 2,Not Applicable
17 Calcineurin Inhibitors Phase 4,Phase 3
18 Vaccines Phase 4,Phase 3,Not Applicable
19 Vitamin B Complex Phase 4,Phase 1,Phase 2
20 Antimalarials Phase 4
21 Fanasil, pyrimethamine drug combination Phase 4
22 Antiparasitic Agents Phase 4
23 Anti-Infective Agents, Urinary Phase 4
24 Vitamin B9 Phase 4,Phase 1,Phase 2
25 Folate Phase 4,Phase 1,Phase 2
26 Folic Acid Antagonists Phase 4
27 Antiprotozoal Agents Phase 4
28 Renal Agents Phase 4
29 Proton Pump Inhibitors Phase 4
30 Gastrointestinal Agents Phase 4,Phase 3,Phase 1,Phase 2,Not Applicable
31 Antacids Phase 4
32 Anti-Ulcer Agents Phase 4
33
Valproic Acid Approved, Investigational Phase 3 99-66-1 3121
34
Lamotrigine Approved, Investigational Phase 3,Phase 2 84057-84-1 3878
35
Etanercept Approved, Investigational Phase 3,Not Applicable 185243-69-0
36
Methylprednisolone Approved, Vet_approved Phase 3,Not Applicable 83-43-2 6741
37
Methylprednisolone hemisuccinate Approved Phase 3,Not Applicable 2921-57-5
38
Prednisolone Approved, Vet_approved Phase 3,Not Applicable 50-24-8 5755
39
Prednisolone phosphate Approved, Vet_approved Phase 3,Not Applicable 302-25-0
40
Pembrolizumab Approved Phase 3 1374853-91-4
41
Hydrochlorothiazide Approved, Vet_approved Phase 3 58-93-5 3639
42
Coal tar Approved Phase 3 8007-45-2
43
Gabapentin Approved, Investigational Phase 2, Phase 3 60142-96-3 3446
44
Acetaminophen Approved Phase 3 103-90-2 1983
45
ofatumumab Approved Phase 3 679818-59-8 6918251
46
Prednisone Approved, Vet_approved Phase 3 53-03-2 5865
47
Cetirizine Approved Phase 3 83881-51-0 2678
48
Histamine Approved, Investigational Phase 3 51-45-6 774
49
Calcium Approved, Nutraceutical Phase 3,Phase 2 7440-70-2 271
50
Prednisolone hemisuccinate Experimental Phase 3,Not Applicable 2920-86-7

Interventional clinical trials:

(show top 50) (show all 54)
# Name Status NCT ID Phase Drugs
1 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
2 A Study of Fluzone® High-Dose Vaccine Compared With Fluzone® Vaccine In Elderly Adults Completed NCT01427309 Phase 4
3 IPT of Malaria With SP in Different Zones of Drug Resistance in Rwanda Completed NCT00372632 Phase 4 Sulfadoxine-Pyrimethamine;placebo
4 Stress Ulcer Prophylaxis in the Intensive Care Unit Completed NCT02467621 Phase 4 Pantoprazole
5 G-CSF in the Treatment of Toxic Epidermal Necrolysis Recruiting NCT02739295 Phase 4 recombinant granulocyte - colony stimulating factor;NaCl 0.9%
6 Phase III Clinical Trial of NPB-01 in Patients With Stevens-Johnson Syndrome/ Toxic Epidermal Necrolysis Unresponsive to Corticosteroids. Completed NCT01696500 Phase 3 Intravenous immunoglobulin
7 Clinical Evaluation of BW430C in Epilepsy Completed NCT00395694 Phase 3 lamictal
8 Autologous ex Vivo Conjunctival Epithelial Cell Expansion for Ocular Surface Transplantation Completed NCT00346450 Phase 3
9 Long-term Safety and Efficacy Extension Study for Participants With Advanced Tumors Who Are Currently on Treatment or in Follow-up in a Pembrolizumab (MK-3475) Study (MK-3475-587/KEYNOTE-587) Recruiting NCT03486873 Phase 3 Standard of Care (SOC)
10 Postpartum Management of Hypertension in Pregnancy With Hydrochlorothiazide Recruiting NCT03298802 Phase 3 Hydrochlorothiazide 50mg Tablet;Placebo Tablet
11 Cyclosporine and Etanercept in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Not yet recruiting NCT02987257 Phase 3 cyclosporin A;Etanercept 50 MG Solution for Injection;Placebos
12 Lamictal in the Treatment of Post-Herpetic Neuralgia Terminated NCT00295776 Phase 2, Phase 3 Lamictal in the treatment of Post-Herpetic Neuralgia
13 Multi-Year Study of Fluzone High-Dose Influenza Vaccine Compared With Fluzone® Vaccine in Adults Aged 65 Years and Older Terminated NCT00976027 Phase 3
14 Long-Term Extension Study of Ofatumumab in Subjects With Pemphigus Vulgaris Terminated NCT02613910 Phase 3 Ofatumumab;Acetaminophen/paracetamol;Antihistamine (cetirizine or equivalent);Prednisone/Prednisolone
15 Cultivated Stem Cell Transplantation for the Treatment of Limbal Stem Cell Deficiency Unknown status NCT00845117 Phase 1, Phase 2
16 Utilization of Amniotic Membrane Extract Eye Drop (AMEED) on Human Corneal Healing Completed NCT02746848 Phase 1, Phase 2
17 Use of Cross-linked Donor Corneas as Carriers for the Boston Keratoprosthesis Completed NCT01582880 Phase 1, Phase 2 Riboflavin
18 A Study of LY2484595 in Japanese Subjects Completed NCT01375075 Phase 2 LY2484595;Placebo;Atorvastatin
19 Limbal Epithelial Stem Cell Transplantation: a Phase II Multicenter Trial Recruiting NCT02318485 Phase 2
20 A Roll-over Study With Rilpivirine for Human Immunodeficiency Virus Type 1 (HIV-1) Infected Participants Who Participated in the Rilpivirine Pediatric Studies Recruiting NCT02494986 Phase 2 Rilpivirine
21 Topical Clobetasol for the Treatment of Toxic Epidermal Necrolysis Active, not recruiting NCT02319616 Phase 1, Phase 2 Clobetasol 0.05% ointment;Placebo
22 Study to Evaluate the Use of Palifermin to Treat Toxic Epidermal Necrolysis Terminated NCT02037347 Phase 1, Phase 2 Palifermin
23 Infliximab to Improve Retention of the Boston Keratoprosthesis in Patients After Stevens Johnson Syndrome/ Toxic Epidermal Necrolysis (SJS/TENS) Withdrawn NCT01256489 Phase 1, Phase 2 Infliximab
24 Study of a Potential New Treatment for Patients With Toxic Epidermal Necrolysis (TEN) Withdrawn NCT00372723 Phase 2 Remicaide (infliximab)
25 Topical Infliximab in Autoimmune Eyes With Keratoprosthesis Withdrawn NCT02126020 Phase 1, Phase 2 topical infliximab
26 Topical Infliximab for Sterile Corneal Melt Recruiting NCT02987686 Phase 1 Topical Infliximab
27 PRF in Management of Chronic Multiple Oral Ulcers Not yet recruiting NCT03878771 Phase 1 Dermovate cream in Orabase
28 Corneal Epitheliotropic Factors in Autologous Serum Eye Drops in Nonautoimmune and Stevens-Johnson Syndrome With Dry Eye Unknown status NCT01122303
29 HLA-B*5801 Screening to Prevent Allopurinol-induced Severe Cutaneous Adverse Reaction Unknown status NCT03046914 Not Applicable
30 Stevens-Johnson Syndrome Antimicrobial Completed NCT00844038
31 Salivary Gland Transplantation in the Treatment of Dry Eye in Patients With Stevens-Johnson Syndrome. Completed NCT01178242 Not Applicable
32 Evaluation of TNF-α Blockade Effect in Patients With Severe Cutaneous Adverse Drug Reactions Completed NCT01276314 Not Applicable anti- TNF-a;Prednisolone
33 Perioperative Research Into Memory: Cognitive Outcome Following Major Burns Completed NCT03470844
34 Immune Responses to Antigens in Non-infectious Eye Inflammatory Diseases Completed NCT00357071
35 Bacteremia Antibiotic Length Actually Needed for Clinical Effectiveness: A Pilot RCT Completed NCT02261506 Not Applicable
36 Safety and Performance Study of the ARGOS-IO System in Patients Undergoing Boston Keratoprosthesis Implantation Completed NCT02945176 Not Applicable
37 Comparative Effectiveness of Intravenous v. Oral Antibiotic Therapy for Serious Bacterial Infections Completed NCT02311452
38 Risk of Hospitalization for Severe Hypersensitivity (Including Severe Skin Reactions) in Patients With Type 2 Diabetes Exposed to Oral Antidiabetic Treatments Completed NCT01086319
39 Post-Licensure Safety Study of ISENTRESS™ (Raltegravir) in a United States Managed Care Network (MK-0518-268) Completed NCT01078246
40 Ocular Safety Of Patients Receiving Macugen For Neovascular Age Related Macular Degeneration Completed NCT00460408 Macugen
41 Evaluating the Effect of Isotretinoin in Regulatory T-cell Function in Adverse Cutaneous Drug Eruptions (ACDEs): A Pilot Study Recruiting NCT02795143 Not Applicable Isotretinoin
42 HLA Screening in Reducing the Risk of Antiepileptic Drug-induced Cutaneous Adverse Reactions Recruiting NCT03184597 Not Applicable
43 Drug Reactions Sampling (COLLECTIONTOXIDERMIES) Recruiting NCT03659227
44 Drug Patch Tests in Patients With Severe Cutaneous Adverse Reaction to Drugs (SCARs) Recruiting NCT03176342 Not Applicable
45 Severe Cutaneous Adverse Reactions in Thailand Recruiting NCT02574988
46 An Exploratory Study of Genetic and Clinical Factors for Serious Skin Reactions Among Users of Eslicarbazepine Acetate Recruiting NCT02520557
47 Minor Salivary Gland Transplantation for Cicatrizing Conjunctivitis Recruiting NCT03839069 Not Applicable
48 A Surveillance Study of Diseases Specified as Adverse Events of Special Interest, of Other Adverse Events Leading to Hospitalisation or Death, and of Meningitis in Children in Africa Prior to Implementation of the RTS,S/AS01E Candidate Recruiting NCT02374450 Not Applicable
49 Pattern of Use and Safety/Effectiveness of Nivolumab in Routine Oncology Practice Recruiting NCT02847728
50 Observational Study of Nivolumab in Patients in Germany With Squamous Cell Carcinoma of the Head and Neck Progressing on or After Platinum-Based Therapy Recruiting NCT03114163

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:

42
Skin, Eye, Salivary Gland, T Cells, Testes

Publications for Stevens-Johnson Syndrome/toxic Epidermal Necrolysis

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

(show top 50) (show all 136)
# Title Authors Year
1
Incidence of Stevens-Johnson syndrome/toxic epidermal necrolysis among new users of different individual drugs in a European population: a case-population study. ( 30298362 )
2019
2
Serum and blister-fluid elevation and decreased epidermal content of HMGB1 protein in drug-induced Stevens Johnson syndrome/toxic epidermal necrolysis. ( 30613954 )
2019
3
Comprehensive assessment of T cell receptor β repertoire in Stevens-Johnson syndrome/toxic epidermal necrolysis patients using high-throughput sequencing. ( 30623817 )
2019
4
British Association of Dermatologists guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in children and young people 2018. ( 30829411 )
2019
5
Development and Validation of a Risk Prediction Model for In-Hospital Mortality Among Patients With Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis-ABCD-10. ( 30840032 )
2019
6
Sutureless amniotic membrane transplantation with cyanoacrylate glue for acute Stevens-Johnson syndrome/toxic epidermal necrolysis. ( 30872140 )
2019
7
Acute generalized exanthematous pustulosis simulating Stevens-Johnson syndrome/toxic epidermal necrolysis associated with the use of vismodegib. ( 29387760 )
2018
8
Severe Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome-beyond skin involvement. ( 29159901 )
2018
9
The association of HLA B*15:02 allele and Stevens-Johnson syndrome/toxic epidermal necrolysis induced by aromatic anticonvulsant drugs in a South Indian population. ( 29076187 )
2018
10
Stevens-Johnson syndrome/toxic epidermal necrolysis and erythema multiforme drug-related hospitalisations in a national administrative database. ( 29387340 )
2018
11
Improving mortality outcomes of Stevens Johnson syndrome/toxic epidermal necrolysis: A regional burns centre experience. ( 29029855 )
2018
12
Cost-effectiveness analysis of HLA-B*58: 01 genetic testing before initiation of allopurinol therapy to prevent allopurinol-induced Stevens-Johnson syndrome/toxic epidermal necrolysis in a Malaysian population. ( 29176400 )
2018
13
Stevens-Johnson syndrome/toxic epidermal necrolysis overlap in a NSCLC patient treated with afatinib. ( 29328518 )
2018
14
Pediatric Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Halted by Etanercept. ( 29421925 )
2018
15
Stevens-Johnson syndrome/toxic epidermal necrolysis associated with zonisamide. ( 29445458 )
2018
16
Selective Use of Cyclosporine for Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis May Exclude Patients with Poor Prognostic Factors. ( 29559345 )
2018
17
A meta-analysis of cyclosporine treatment for Stevens-Johnson syndrome/toxic epidermal necrolysis. ( 29636627 )
2018
18
Diverse expression of TNF-α and CCL27 in serum and blister of Stevens-Johnson syndrome/toxic epidermal necrolysis. ( 29713456 )
2018
19
Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: A Multicenter Retrospective Study of 377 Adult Patients from the United States. ( 29758282 )
2018
20
A multivariate genetic analysis confirms rs5010528 in the human leucocyte antigen-C locus as a significant contributor to Stevens-Johnson syndrome/toxic epidermal necrolysis susceptibility in a Mozambique HIV population treated with nevirapine. ( 29762688 )
2018
21
Procalcitonin as a diagnostic indicator for systemic bacterial infections in patients with Stevens-Johnson syndrome/toxic epidermal necrolysis. ( 29897140 )
2018
22
Reply to: "Wound management strategies in Stevens-Johnson syndrome/toxic epidermal necrolysis: An unmet need". ( 29913260 )
2018
23
Wound management strategies in Stevens-Johnson syndrome/toxic epidermal necrolysis: An unmet need. ( 29913262 )
2018
24
Stevens-Johnson syndrome/toxic epidermal necrolysis in Jewish and Arab populations. ( 29972250 )
2018
25
Carbamazepine-induced Stevens-Johnson syndrome/toxic epidermal necrolysis overlap in a Filipino with positive HLA-B75 serotype. ( 30018035 )
2018
26
ISMP Adverse Drug Reactions: Propofol-Related Infusion Syndrome (PRIS)1,2; Ivermectin-Induced Stevens-Johnson Syndrome; Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis From Fexofenadine; Memantine-Related Drug Eruption. ( 30038437 )
2018
27
Tolerance Induction to Antituberculosis Drugs in a Patient With Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Overlap. ( 30083412 )
2018
28
Defining Regional Differences in Drug-Induced Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: A Tool to Improve Drug Safety? ( 30120778 )
2018
29
Association between HLA-B*5901 and methazolamide-induced Stevens-Johnson syndrome/toxic epidermal necrolysis: a systematic review and meta-analysis. ( 30242287 )
2018
30
Novel complication of Flakka: Stevens-Johnson syndrome/Toxic epidermal necrolysis overlap. ( 30503281 )
2018
31
Research Directions in Genetic Predispositions to Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis. ( 29105735 )
2018
32
Survey of Nonprescription Medication and Antibiotic Use in Patients with Stevens-Johnson Syndrome, Toxic Epidermal Necrolysis, and Overlap Syndrome. ( 29389866 )
2018
33
The use of cyclosporine for Stevens-Johnson syndrome-toxic epidermal necrolysis spectrum at the University of Louisville: A case series and literature review. ( 29469760 )
2018
34
Management of Stevens-Johnson Syndrome-Toxic Epidermal Necrolysis: Looking Beyond Guidelines! ( 29692452 )
2018
35
Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis and Treatment With a Biologic: A Case Report. ( 28488978 )
2017
36
Genetics and Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: What Have We Learned? ( 28278317 )
2017
37
Effectiveness, safety and tolerability of cyclosporine versus supportive treatment in Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: A record-based study. ( 28366923 )
2017
38
Association of the HLA-B alleles with carbamazepine-induced Stevens-Johnson syndrome/toxic epidermal necrolysis in the Javanese and Sundanese population of Indonesia: the important role of the HLA-B75 serotype. ( 29053440 )
2017
39
What's beneath the surface? Stevens-Johnson syndrome/toxic epidermal necrolysis combined with haemophagocytic lymphohistiocytosis: a case report. ( 28072947 )
2017
40
Cyclosporine treatment for Stevens-Johnson syndrome/toxic epidermal necrolysis: Retrospective analysis of a cohort treated in a specialized referral center. ( 27717620 )
2017
41
Long-term complications of Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN): the spectrum of chronic problems in patients who survive an episode of SJS/TEN necessitates multidisciplinary follow-up. ( 28144971 )
2017
42
Letter to the Editor concerning the article "Long term complications of Stevens-Johnson syndrome/Toxic epidermal necrolysis: The spectrum of chronic problems in patients who survive an episode of SJS/TEN necessitates multi-disciplinary follow up. ( 28626892 )
2017
43
"Microbiological findings and antibacterial therapy in Stevens-Johnson syndrome/Toxic Epidermal Necrolysis patients from a Swedish Burn Center". ( 28075032 )
2017
44
Piperacillin-tazobactam-induced linear IgA bullous dermatosis presenting clinically as Stevens-Johnson syndrome/toxic epidermal necrolysis overlap. ( 28084616 )
2017
45
Serum IL-17 in patients with erythema multiforme or Stevens-Johnson syndrome/toxic epidermal necrolysis drug reaction, and correlation with disease severity. ( 28940568 )
2017
46
Are Idiopathic Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Related to Drugs in Food? The Example of Phenylbutazone. ( 28108296 )
2017
47
Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Overlap Induced by Fexofenadine. ( 28570227 )
2017
48
Plasma Lipid Profiling of Patients with Chronic Ocular Complications Caused by Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis. ( 27898730 )
2016
49
Acute and Chronic Ophthalmic Involvement in Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis - A Comprehensive Review and Guide to Therapy. II. Ophthalmic Disease. ( 26882981 )
2016
50
Guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis: An Indian perspective. ( 27679410 )
2016

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 48)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.86 CCL17 CCL22 CCL5 CCR3 CXCR3 CXCR4
2
Show member pathways
13.64 CCL17 CCL22 CCL5 CCR3 CXCR3 CXCR4
3
Show member pathways
13.53 CCL17 CCL22 CCL5 CXCR4 F2RL2 IFNG
4
Show member pathways
13.35 CCL22 CCL5 HLA-A HLA-B HLA-C IFNG
5
Show member pathways
13.29 CCL17 CCL22 CCL5 CCR3 CXCR3 CXCR4
6
Show member pathways
13.2 CCL17 CCL22 CCL5 CCR3 CXCR3 CXCR4
7
Show member pathways
12.96 CCL5 CCR3 CXCR4 FAS HLA-A HLA-B
8
Show member pathways
12.88 CCL17 CCL22 CCL5 CCR3 CXCR3 CXCR4
9 12.84 CXCR4 FAS IFNG IL13 IL2 IL5
10
Show member pathways
12.71 CXCR4 FAS HLA-A HLA-B HLA-C IFNG
11 12.52 CCL5 CXCR4 FAS IFNG IKZF1 IL2
12
Show member pathways
12.5 FAS HLA-A HLA-B HLA-C IFNG IL2
13
Show member pathways
12.49 HLA-A HLA-B HLA-C IFNG
14
Show member pathways
12.42 CXCR4 IFNG IL13 IL2 IL5
15
Show member pathways
12.41 FAS IFNG IL2 IL5
16
Show member pathways
12.39 CCL5 FAS HLA-A HLA-B HLA-C IFNG
17 12.38 CXCR4 HLA-A HLA-B HLA-C
18 12.34 HLA-A HLA-B HLA-C IL2
19
Show member pathways
12.32 CCL17 IFNG IL13 IL5
20 12.29 CCR3 HLA-A HLA-B HLA-C
21
Show member pathways
12.23 HLA-A HLA-B HLA-C IFNG IL13 IL2
22
Show member pathways
12.22 CCL17 CCL22 CCL5 CCR3 CXCR3 CXCR4
23
Show member pathways
12.21 CCL17 CCL22 IL2 TLR9
24
Show member pathways
12.08 CCL17 CCL22 CCL5 IFNG IL13 IL2
25
Show member pathways
12.04 IL13 IL2 IL5 TLR9
26 12 CCR3 IFNG IL2 IL5
27
Show member pathways
11.93 HLA-A IFNG IL2
28
Show member pathways
11.91 CCL17 CCL22 CCL5 CCR3 CXCR3 CXCR4
29 11.83 IFNG IL13 IL2 IL5
30
Show member pathways
11.79 IFNG IKZF1 IL2 IL5
31 11.77 IFNG IKZF1 IL2 TLR9
32 11.69 CCL5 CCR3 IL13 IL5
33
Show member pathways
11.64 HLA-A IFNG IL2
34 11.56 FAS IFNG IL2
35 11.54 CCL22 CCL5 IL13
36 11.54 CXCR3 IFNG IKZF1 IL13 IL2 IL5
37 11.53 CXCR4 IKZF1 IL5
38 11.41 FAS IFNG TLR9
39 11.35 IFNG IL2 IL5
40 11.33 IFNG IL13 IL5
41 11.3 IFNG IL13 IL2 IL5
42 11.28 CCL17 CCL22 CCL5 IFNG IL13 IL2
43 11.25 IFNG IL13 IL2
44 11.23 IFNG IL13 IL2 IL5
45 11.2 HLA-A HLA-B HLA-C
46 11.2 CCL17 CCL22 CCL5 IL13 IL2 IL5
47 11.04 CCL17 CCL22 CCL5 IL13 IL2 IL5
48 10.76 CCL17 CCL22 IFNG IL13 IL2 IL5

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 extracellular region GO:0005576 9.85 CCL17 CCL22 CCL5 F2RL2 FAS IFNG
2 cell surface GO:0009986 9.73 CXCR3 CXCR4 FAS HLA-A HLA-B HLA-C
3 recycling endosome membrane GO:0055038 9.58 HLA-A HLA-B HLA-C
4 phagocytic vesicle membrane GO:0030670 9.54 HLA-A HLA-B HLA-C
5 ER to Golgi transport vesicle membrane GO:0012507 9.5 HLA-A HLA-B HLA-C
6 integral component of lumenal side of endoplasmic reticulum membrane GO:0071556 9.13 HLA-A HLA-B HLA-C
7 MHC class I protein complex GO:0042612 8.8 HLA-A HLA-B HLA-C

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

(show all 33)
# Name GO ID Score Top Affiliating Genes
1 regulation of signaling receptor activity GO:0010469 9.98 CCL17 CCL22 CCL5 IFNG IL13 IL2
2 cytokine-mediated signaling pathway GO:0019221 9.97 CCL22 CCL5 IL13 IL2 IL5
3 cell-cell signaling GO:0007267 9.92 CCL17 CCL22 CCL5 IL2
4 positive regulation of cytosolic calcium ion concentration GO:0007204 9.9 CCR3 CXCR3 CXCR4 IL2
5 response to virus GO:0009615 9.85 CCL22 CCL5 CXCR4 IFNG
6 cellular response to interferon-gamma GO:0071346 9.82 CCL17 CCL22 CCL5
7 calcium-mediated signaling GO:0019722 9.81 CCR3 CXCR3 CXCR4
8 neutrophil chemotaxis GO:0030593 9.81 CCL17 CCL22 CCL5
9 cellular response to interleukin-1 GO:0071347 9.8 CCL17 CCL22 CCL5
10 interferon-gamma-mediated signaling pathway GO:0060333 9.8 HLA-A HLA-B HLA-C IFNG
11 type I interferon signaling pathway GO:0060337 9.79 HLA-A HLA-B HLA-C
12 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.78 CCL5 IFNG IL13 IL2
13 monocyte chemotaxis GO:0002548 9.77 CCL17 CCL22 CCL5
14 antigen processing and presentation GO:0019882 9.76 HLA-A HLA-B HLA-C
15 lymphocyte chemotaxis GO:0048247 9.75 CCL17 CCL22 CCL5
16 antigen processing and presentation of peptide antigen via MHC class I GO:0002474 9.73 HLA-A HLA-B HLA-C
17 cell chemotaxis GO:0060326 9.72 CCL22 CCL5 CCR3 CXCR3 CXCR4
18 antigen processing and presentation of exogenous peptide antigen via MHC class I, TAP-dependent GO:0002479 9.71 HLA-A HLA-B HLA-C
19 positive regulation of B cell proliferation GO:0030890 9.71 IL13 IL2 IL5 TLR9
20 positive regulation of T cell mediated cytotoxicity GO:0001916 9.69 HLA-A HLA-B HLA-C
21 dendritic cell chemotaxis GO:0002407 9.66 CCL5 CXCR4
22 regulation of regulatory T cell differentiation GO:0045589 9.65 IFNG IL2
23 chemokine-mediated signaling pathway GO:0070098 9.65 CCL17 CCL22 CCL5 CCR3 CXCR3
24 positive regulation of immunoglobulin secretion GO:0051024 9.64 IL2 IL5
25 antigen processing and presentation of exogenous peptide antigen via MHC class I, TAP-independent GO:0002480 9.63 HLA-A HLA-B HLA-C
26 chemotaxis GO:0006935 9.63 CCL17 CCL22 CCL5 CCR3 CXCR3 CXCR4
27 positive regulation of immunoglobulin production GO:0002639 9.62 IL13 TLR9
28 protection from natural killer cell mediated cytotoxicity GO:0042270 9.61 HLA-A HLA-B
29 inflammatory response GO:0006954 9.61 CCL17 CCL22 CCL5 CCR3 CXCR3 CXCR4
30 antigen processing and presentation of endogenous peptide antigen via MHC class Ib GO:0002476 9.54 HLA-A HLA-B HLA-C
31 immune response GO:0006955 9.5 CCL17 CCL22 CCL5 CCR3 CXCR3 CXCR4
32 G protein-coupled receptor signaling pathway GO:0007186 10.13 CCL17 CCL22 CCL5 CCR3 CXCR3 CXCR4
33 immune system process GO:0002376 10.05 HLA-A HLA-B HLA-C IL2 TLR9

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

# Name GO ID Score Top Affiliating Genes
1 chemokine activity GO:0008009 9.63 CCL17 CCL22 CCL5
2 peptide antigen binding GO:0042605 9.61 HLA-A HLA-B HLA-C
3 CCR chemokine receptor binding GO:0048020 9.58 CCL17 CCL22 CCL5
4 chemokine binding GO:0019956 9.5 CCR3 CXCR3 CXCR4
5 C-X-C chemokine receptor activity GO:0016494 9.48 CXCR3 CXCR4
6 C-C chemokine binding GO:0019957 9.43 CCR3 CXCR3 CXCR4
7 CCR4 chemokine receptor binding GO:0031729 9.37 CCL17 CCL5
8 C-C chemokine receptor activity GO:0016493 9.33 CCR3 CXCR3 CXCR4
9 cytokine activity GO:0005125 9.17 CCL17 CCL22 CCL5 IFNG IL13 IL2
10 chemokine receptor activity GO:0004950 9.13 CCR3 CXCR3 CXCR4

Sources for Stevens-Johnson Syndrome/toxic Epidermal Necrolysis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
Content
Loading form....