| H01694 | |
| H番号 | H01694 |
| 名称 | スティーブンス・ジョンソン症候群; 中毒性表皮壊死症; ライエル症候群; 重症多形滲出性紅斑 |
| 概要 | 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. |
| カテゴリ | 免疫系疾患; 皮膚疾患 |
| ネットワーク | - |
| 病因遺伝子 | HLA-A (polymorphism) [HSA:3105] [KO:K06751] HLA-B (polymorphism) [HSA:3106] [KO:K06751] |
| 病原体 | - |
| 環境要因 | - |
| 発癌物質 | - |
| 治療薬 | デキサメタゾン [DR:D00292] デキサメタゾンリン酸エステルナトリウム [DR:D00975] ベタメタゾンリン酸エステルナトリウム [DR:D00972] トリアムシノロンアセトニド [DR:D00983] 精製ヒアルロン酸ナトリウム [DR:D03354] ヒドロコルチゾン [DR:D00088] ヒドロコルチゾンコハク酸エステルナトリウム [DR:D00978] コルチゾン酢酸エステル [DR:D00973] トリアムシノロン [DR:D00385] ベタメタゾン [DR:D00244] プレドニゾロン [DR:D00472] プレドニゾロンコハク酸エステルナトリウム [DR:D01239] メチルプレドニゾロン [DR:D00407] メチルプレドニゾロン酢酸エステル [DR:D00979] 乾燥ポリエチレングリコール処理人免疫グロブリン [DR:D08788] |
| コメント | Two genetic risk variants, HLA-B1502 and HLA-A3101, for Carbamazepine-induced hypersensitivity reactions have been identified. |
| リンク | ICD-11: EB13 ICD-10: L51.1 L51.2 MeSH: D013262 OMIM: 608579 |
| 文献 | PMID:27738400 著者 Cekic S, Canitez Y, Sapan N タイトル Evaluation of the patients diagnosed with Stevens Johnson syndrome and toxic epidermal necrolysis: a single center experience. 雑誌 Turk Pediatri Ars 51:152-158 (2016) DOI:10.5152/TurkPediatriArs.2016.3836 PMID:24597466 著者 Amstutz U, Shear NH, Rieder MJ, Hwang S, Fung V, Nakamura H, Connolly MB, Ito S, Carleton BC タイトル Recommendations for HLA-B*15:02 and HLA-A*31:01 genetic testing to reduce the risk of carbamazepine-induced hypersensitivity reactions. 雑誌 Epilepsia 55:496-506 (2014) DOI:10.1111/epi.12564 |