{"orpha":88633,"name":"Superior limbic keratoconjunctivitis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["SLK","Theodore superior limbic keratoconjunctivitis","Theodore syndrome"],"definition":"A rare disorder of the anterior segment of the eye characterized by unilateral or bilateral, chronic and recurrent inflammation affecting the upper tarsal and bulbar conjunctiva, as well as the superior limbus, manifesting as a papillary reaction on the upper tarsal conjunctiva, thickening and folding of redundant superior bulbar conjunctiva, and superficial punctate epithelial keratitis with or without filament formation near the superior corneal limbus. Middle-aged women are most commonly affected and present with foreign body sensation, frequent blinking, burning sensation, and pruritus, among others.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-88633/","orphanet_url":"https://www.orpha.net/en/disease/detail/88633","names":{"fr":{"name":"Kératoconjonctivite limbique supérieure","synonyms":["Kératoconjonctivite limbique supérieure de Théodore","Syndrome de Théodore"],"definition":"Maladie rare du segment antérieur de l'oeil caractérisée par une inflammation unilatérale ou bilatérale, chronique et récurrente, affectant la conjonctive supérieure tarsale et bulbaire, ainsi que le limbe supérieur, se manifestant par une conjonctivite papillaire sur la conjonctive tarsienne supérieure, un épaississement et un prolapsus de la conjonctive tarsienne supérieure redondante, et une kératite épithéliale ponctuée superficielle avec ou sans formation de filaments près du limbe cornéen supérieur. Les femmes d'âge moyen sont le plus souvent touchées et se plaignent, entre autres, d'une sensation de corps étranger, des clignements fréquents, d'une sensation de brûlure et d'un prurit."},"de":{"name":"Keratokonjunktivitis, limbische superiore","synonyms":["SLK","Superiore limbische Keratokonjunktivitis Theodore","Theodore-Syndrom"],"definition":"Eine seltene Erkrankung des vorderen Augenabschnitts, die durch eine chronische, rezidivierende, ein- oder beidseitige Entzündung der oberen tarsalen und bulbären Bindehaut sowie des oberen Limbus gekennzeichnet ist und sich durch eine papilläre Reaktion der oberen Bindehaut, eine Verdickung und Fältelung der überflüssigen oberen Bindehaut und eine oberflächliche, punktförmige Epithelkeratitis mit oder ohne Fadenbildung in der Nähe des oberen Hornhautlimbus manifestiert. Am häufigsten sind Frauen mittleren Alters betroffen, die u.a. ein Fremdkörpergefühl, häufiges Blinzeln, Brennen und Juckreiz angeben."},"es":{"name":"Queratoconjuntivitis límbica superior","synonyms":["Queratoconjuntivitis límbica superior de Theodore","SLK","Síndrome de Theodore"],"definition":"Es un trastorno poco frecuente del segmento anterior del ojo caracterizado por inflamación uni- o bilateral, crónica y recurrente, que afecta a la conjuntiva bulbar y tarsal superior así como al limbo superior, que se manifiesta como una reacción papilar en la conjuntiva tarsal superior, engrosamiento y plegamiento de la conjuntiva bulbar superior redundante y queratitis punteada superficial con o sin formación de filamentos cerca del limbo corneal superior. Afecta mayoritariamente a mujeres de mediana edad y se manifiesta como una sensación de cuerpo extraño, parpadeo frecuente, ardor y prurito, entre otros."},"it":{"name":"Cheratocongiuntivite limbica superiore","synonyms":["Cheratocongiuntivite limbica superiore di Theodore","SLK","Sindrome di Theodore"],"definition":"Si tratta di una malattia rara del segmento anteriore dell'occhio, caratterizzata da una infiammazione cronica ricorrente, mono- o bilaterale, della congiuntiva tarsale superiore e bulbare e del limbus superiore, che si manifesta sotto forma di reazione papillare sulla congiuntiva tarsale superiore, ispessimento e ripiegamento della congiuntiva bulbare superiore ridondante e cheratite epiteliale puntata superficiale con o senza formazione di filamenti in prossimità del limbus corneale superiore. Le donne di mezza età sono maggiormente colpite e presentano una sensazione di corpo estraneo, frequenti battiti delle palpebre, bruciore e prurito."},"nl":{"name":"Superieure limbische keratoconjunctivitis","synonyms":["SLK","Superieure limbische keratoconjunctivitis van Theodore","Syndroom van Theodore"],"definition":"Een zeldzame aandoening van voorste oogsegment, gekarakteriseerd door unilaterale of bilaterale, chronische en recidiverende inflammatie van bovenste conjunctiva palpebrarum en bulbaire conjunctiva, alsook van limbus superior, die zich manifesteert als papillaire reactie aan bovenste conjunctiva palpebrarum, verdikking en plooiing van overtollig bovenste bulbaire conjunctiva, en oppervlakkige epitheliale keratitis punctata met of zonder vorming van filament nabij limbus corneae superior. Vrouwen van middelbare leeftijd worden het vaakst getroffen en presenteren zich met onder meer gevoel van corpus alienum, frequent knipperen met ogen, brandend gevoel, en pruritus."},"pt":{"name":"Queratoconjuntivite límbica superior"},"pl":{"name":"Górne rąbkowe zapalenie spojówki i rogówki","synonyms":["Górne rąbkowe zapalenie spojówki i rogówki Theodore'a","SLK","Zespół Theodore'a"]},"cs":{"name":"Horní limbická keratokonjunktivitida","synonyms":["SLK","Theodorova horní limbická keratokonjunktivitida","Theodorův syndrom"]},"uk":{"name":"Верхній лімбічний кератоконюнктивіт","synonyms":["SLK","Верхній лімбічний кератоконюнктивіт Теодора","Синдром Теодора"],"definition":"A rare disorder of the anterior segment of the eye characterized by unilateral or bilateral, chronic and recurrent inflammation affecting the upper tarsal and bulbar conjunctiva, as well as the superior limbus, manifesting as a papillary reaction on the upper tarsal conjunctiva, thickening and folding of redundant superior bulbar conjunctiva, and superficial punctate epithelial keratitis with or without filament formation near the superior corneal limbus. Middle-aged women are most commonly affected and present with foreign body sensation, frequent blinking, burning sensation, and pruritus, among others."},"zh":{"name":"上角膜缘角膜结膜炎","synonyms":["Theodore上缘角膜结膜炎"]},"tr":{"name":"Süperior limbik keratokonjunktivit","synonyms":["SLK","Theodore sendromu","Theodore süperior limbik keratokonjunktivit"]}},"xrefs":[{"source":"ICD-10","code":"H16.2","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"9A60.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1962803547"},{"source":"MONDO","code":"0019504","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0339229","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"10940","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10078410","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97966,"name":"Rare ophthalmic disorder"},"parents":[{"orpha":519280,"name":"Rare conjunctivitis","classifications":[189]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}