KEGG   DISEASE: Stevens-Johnson syndrome
Entry
H01694                      Disease                                

Name
Stevens-Johnson syndrome;
Toxic epidermal necrolysis;
Lyell syndrome
Description
Stevens Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe acute mucocutaneous diseases. The early stage of the disease is characterized by red-purple maculopapular eruptions. Then epidermal separation occurs and vesicles and bullae are formed. Inflammatory changes including purulent conjunctivitis, erosion, ulcer and crusts may be observed in the eye, mouth, nose, pharynx, esophagus, trachea, gastrointestinal tract, urinary tract and genital mucosae. Life-threatening bleeding and infections may be observed as a result of these changes. The rates of severe complications or sequelae secondary to SJS and TEN are higher in patients with mucosal and ophthalmic involvement. The SJS and TEN are considered the same disease process, and the distinction is made based on body surface area involvement. The SJS is characterized by less than 10% of the body surface area of epidermal detachment, and TEN by more than 30%. Various etiologic factors have been implicated as causes of SJS-TEN. These include infection, vaccination, drugs, systemic diseases, physical agents, and food. Drugs are the most commonly blamed. It has been reported that SJS-TEN is strongly associated with the specific variants of the human leukocyte antigen HLA-A and HLA-B genes. There is still no consensus on a definite treatment method for SJS-TEN. Systemic steroids and IVIG are used most frequently in medical treatment and treatment options including cyclosporine, plasmapheresis and hemodialysis are required more rarely.
Category
Skin and connective tissue disease
Brite
Human diseases [BR:br08402]
 Skin diseases
  Skin and soft tissue diseases
   H01694  Stevens-Johnson syndrome
Human diseases in ICD-11 classification [BR:br08403]
 14 Diseases of the skin
  Inflammatory dermatoses
   Inflammatory erythemas and other reactive inflammatory dermatoses
    EB13  Stevens-Johnson syndrome or toxic epidermal necrolysis
     H01694  Stevens-Johnson syndrome
Gene
HLA-A (polymorphism) [HSA:3105] [KO:K06751]
HLA-B (polymorphism) [HSA:3106] [KO:K06751]
Env factor
Carbamazepine, Aminopenicillins, Cephalosporins, Macrolides, Tetracyclines, Quinolones
Drug
Triamcinolone acetonide [DR:D00983]
Dexamethasone [DR:D00292]
Dexamethasone sodium phosphate [DR:D00975]
Hydrocortisone [DR:D00088]
Hydrocortisone sodium succinate [DR:D00978]
Prednisolone [DR:D00472]
Prednisolone sodium phosphate [DR:D00981]
Prednisone [DR:D00473] (Stevens-Johnson syndrome)
Methylprednisolone [DR:D00407]
Methylprednisolone sodium succinate [DR:D00751]
Methylprednisolone acetate [DR:D00979]
Corticotropin [DR:D00146]
Cortisone acetate [DR:D00973]
Comment
Two genetic risk variants, HLA-B1502 and HLA-A3101, for Carbamazepine-induced hypersensitivity reactions have been identified.
Other DBs
ICD-11: EB13
ICD-10: L51.1 L51.2
MeSH: D013262
OMIM: 608579
Reference
  Authors
Cekic S, Canitez Y, Sapan N
  Title
Evaluation of the patients diagnosed with Stevens Johnson syndrome and toxic epidermal necrolysis: a single center experience.
  Journal
Turk Pediatri Ars 51:152-158 (2016)
DOI:10.5152/TurkPediatriArs.2016.3836
Reference
  Authors
Amstutz U, Shear NH, Rieder MJ, Hwang S, Fung V, Nakamura H, Connolly MB, Ito S, Carleton BC
  Title
Recommendations for HLA-B*15:02 and HLA-A*31:01 genetic testing to reduce the risk of carbamazepine-induced hypersensitivity reactions.
  Journal
Epilepsia 55:496-506 (2014)
DOI:10.1111/epi.12564
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