{"orpha":163934,"name":"Atopic keratoconjunctivitis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare, chronic allergic disease of the cornea and conjunctiva occurring in all age groups, characterized by severe itching and burning sensation, conjunctival injection, photophobia and edema with serious cases leading to ulceration of the cornea which can result in blindness. It is often associated with atopic dermatitis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-163934/","orphanet_url":"https://www.orpha.net/en/disease/detail/163934","names":{"fr":{"name":"Kératoconjonctivite atopique","definition":"Maladie allergique chronique rare de la cornée et de la conjonctive touchant tous les groupes d'âge. Elle se caractérise par de fortes démangeaisons et une sensation de brûlure, une injection conjonctivale, une photophobie et un oedème. Les cas graves peuvent entraîner une ulcération de la cornée pouvant conduire à la cécité. Une dermatite atopique est souvent associée."},"de":{"name":"Keratokonjunktivitis, atopische","definition":"Die Atopische Kerato-Konjunktivitis ist eine seltene, chronisch-allergische Erkrankung der Kornea und Konjunktiva. Sie tritt in allen Altersgruppen auf und ist gekennzeichnet durch schweren Juckreiz und brennendes Gefühl, Rötung der Konjunktiva, Photophobie und Ödem, in schweren Fällen mit Ulzeration der Kornea und möglicher Erblindung. Oft besteht gleichzeitig eine atopische Dermatitis."},"es":{"name":"Queratoconjuntivitis atópica","definition":"Es una enfermedad alérgica crónica y poco frecuente de la córnea y la conjuntiva que se da en todos los grupos de edad y que se caracteriza por una picazón intensa y sensación de ardor, inyección conjuntival, fotofobia y edema con casos graves que dan lugar a ulceraciones de la córnea que pueden resultar en ceguera. Se asocia a menudo con la dermatitis atópica."},"it":{"name":"Cheratocongiuntivite atopica","definition":"La cheratocongiuntivite atopica è una malattia allergica cronica rara della cornea e della congiuntiva, che può insorgere a tutte le età. È caratterizzata da una forte sensazione di prurito e bruciore, con iniezione congiuntivale, fotofobia ed edema che, nei casi gravi, può esitare nell'ulcera corneale ed, eventualmente, provocare la cecità. Si associa spesso alla dermatite atopica."},"nl":{"name":"Atopische keratoconjunctivitis","definition":"Een zeldzame, chronische allergische ziekte van cornea en conjunctiva bij alle leeftijdsgroepen, gekarakteriseerd door ernstige jeuk en branderig gevoel, hyperemie van conjunctiva, fotofobie en oedeem dat in ernstige gevallen leidt tot ulceratie van het hoornvlies met mogelijk blindheid tot gevolg. Het is vaak geassocieerd met atopische dermatitis."},"pt":{"name":"Queratoconjuntivite atópica"},"pl":{"name":"Atopowe zapalenie spojówek i rogówki"},"cs":{"name":"Atopická keratokonjunktivitida"},"uk":{"name":"Атопічний кератоконюнктивіт","definition":"A rare, chronic allergic disease of the cornea and conjunctiva occurring in all age groups, characterized by severe itching and burning sensation, conjunctival injection, photophobia and edema with serious cases leading to ulceration of the cornea which can result in blindness. It is often associated with atopic dermatitis."},"zh":{"name":"特应性角膜结膜炎","definition":"一种罕见的慢性过敏性角膜结膜疾病，可发生于所有年龄段，其特征是严重的瘙痒和灼烧感，结膜充血，畏光和水肿，严重情况下会导致角膜溃疡，造成失明。该病常伴特应性皮炎。"},"tr":{"name":"Atopik keratokonjunktivit"}},"xrefs":[{"source":"ICD-10","code":"H16.2","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"9A60.0Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1941631830"},{"source":"MONDO","code":"0015599","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1274788","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10069664","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":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-5 / 10 000","value":15.0,"area":"Europe","status":"Validated","source":"European Medicines Agency 2009[OTHER]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2023-09-12","source":"26278858[PMID]_28846256[PMID]","terms":[{"hpo":"HP:0007957","label":"Corneal opacity","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000958","label":"Dry skin","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000491","label":"Keratitis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001097","label":"Keratoconjunctivitis sicca","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000492","label":"Abnormal eyelid morphology","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0007879","label":"Allergic conjunctivitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000498","label":"Blepharitis","frequency":"Frequent (79-30%)","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:0011496","label":"Corneal neovascularization","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011457","label":"Loss of eyelashes","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0200026","label":"Ocular pain","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000613","label":"Photophobia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012375","label":"Chemosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000559","label":"Corneal scarring","frequency":"Occasional (29-5%)","criteria":null}]}}