{"orpha":137599,"name":"Herpes simplex virus stromal keratitis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Herpes simplex (HSV) stromal keratitis is an infectious ocular disease of either necrotizing or non-necrotizing form, due to an HSV infection, and characterized by corneal stromal necrosis, inflammation, ulceration and infiltration by leukocytes. Corneal perforation and blindness can also occur in severe cases.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-137599/","orphanet_url":"https://www.orpha.net/en/disease/detail/137599","names":{"fr":{"name":"Kératite stromale à herpes simplex","synonyms":["Kératite stromale herpétique"],"definition":"Maladie oculaire infectieuse pouvant prendre une forme nécrosante ou non nécrosante, due à une infection par le virus herpes simplex et caractérisée par une nécrose du stroma cornéen, une inflammation, une ulcération et une infiltration leucocytaire. Dans les cas graves, elle peut également entraîner une perforation de la cornée et la cécité."},"de":{"name":"Herpes simplex-Stromakeratitis","synonyms":["HSV-Stromakeratitis","Stromakeratitis, herpetische"],"definition":"Die Herpes simplex (HSV)-Stromakeratitis ist eine infektiöse Augenerkrankung und tritt in nekrotisierender oder nicht-nekrotisierender Form auf. Ursache ist eine HSV-Infektion. Merkmale sind Nekrose des Korneastromas, Entzündung, Ulzeration und Leukozyten-Infiltrate. In schweren Fällen perforiert die Kornea, die Patienten können erblinden."},"es":{"name":"Queratitis estromal","definition":"La queratitis estromal por herpes simple (HSV) es una enfermedad ocular infecciosa de forma necrotizante o no necrotizante, debida a una infección por HSV, y que se caracteriza por una necrosis estromal corneal, inflamación, ulceración e infiltración leucocitaria. En casos graves puede darse perforación corneal y ceguera."},"it":{"name":"Cheratite stromale","definition":"La cheratite stromale da virus dell'Herpes simplex (HSV) è una malattia oculare infettiva, dovuta all'infezione da HSV. Si differenzia in una forma necrotizzante e in una forma non necrotizzate. È caratterizzata da necrosi stromale corneale, infiammazione, ulcera e infiltrazione leucocitaria. Nei casi gravi si verifica la perforazione corneale e quindi la cecità."},"nl":{"name":"Herpes-simplexvirus stromale keratitis","definition":"Een infectieuze aandoening van oog, aanwezig in een necrotiserende of niet-necrotiserende vorm, veroorzaakt door een HSV-infectie, en gekenmerkt door necrose, inflammatie, ulceratie en infiltratie door leukocyten van stroma van cornea. Perforatie van hoornvlies en blindheid kunnen ook optreden in ernstige gevallen."},"pt":{"name":"Queratite do estroma por herpes simplex virus"},"pl":{"name":"Zapalenie zrębu rogówki"},"cs":{"name":"Stromální keratitida"},"uk":{"name":"Стромальний кератит, спричинений вірусом простого герпесу","definition":"Herpes simplex (HSV) stromal keratitis is an infectious ocular disease of either necrotizing or non-necrotizing form, due to an HSV infection, and characterized by corneal stromal necrosis, inflammation, ulceration and infiltration by leukocytes. Corneal perforation and blindness can also occur in severe cases."},"zh":{"name":"单纯疱疹病毒角膜基质炎","definition":"单纯疱疹病毒(HSV)间质性角膜炎是一种由单纯疱疹病毒感染引起的坏死性或非坏死性的感染性眼部疾病，其特征为角膜间质坏死、炎症、溃疡和白细胞浸润。严重的病例还可能发生角膜穿孔和失明。"},"tr":{"name":"Herpes simpleks virüsü stromal keratiti"}},"xrefs":[{"source":"ICD-10","code":"H16.3","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"1F00.10","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1665288755"},{"source":"MONDO","code":"0015291","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1301533","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"19879","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97966,"name":"Rare ophthalmic disorder"},"parents":[{"orpha":519278,"name":"Infective keratitis","classifications":[189]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":4.2091,"area":"Worldwide","status":"Validated","source":"32732703[PMID]_34622738[PMID]_33607690[PMID]_3391390[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":5.4,"area":"France","status":"Validated","source":"22944008[PMID]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2023-09-12","source":"31424862[PMID]_26989540[PMID]_27221681[PMID]","terms":[{"hpo":"HP:0012040","label":"Corneal stromal edema","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0007765","label":"Deep anterior chamber","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012108","label":"Open angle glaucoma","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0007663","label":"Reduced visual acuity","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000622","label":"Blurred vision","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030953","label":"Conjunctival hyperemia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100583","label":"Corneal perforation","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012155","label":"Decreased corneal sensation","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012039","label":"Descemet Membrane Folds","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0009926","label":"Epiphora","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0007812","label":"Herpetiform corneal ulceration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0031448","label":"Herpetiform vesicles","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0007906","label":"Ocular hypertension","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000618","label":"Blindness","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000491","label":"Keratitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011134","label":"Low-grade fever","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0033834","label":"Malaise","frequency":"Occasional (29-5%)","criteria":null}]}}