{"orpha":519408,"name":"Mooren ulcer","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare disorder of the anterior segment of the eye characterized by a unilateral or bilateral rapidly progressive, intractable, painful, ulcerative keratitis which initially affects the peripheral cornea and may spread circumferentially and then centrally. The destructive process involves stromal corneal tissue only, leaving the epithelium and endothelium largely unaffected. There is no involvement of the adjacent sclera. The condition can be complicated by glaucoma, cataract, and perforation.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-519408/","orphanet_url":"https://www.orpha.net/en/disease/detail/519408","names":{"fr":{"name":"Ulcère de Mooren","definition":"Maladie rare du segment antérieur de l'oeil caractérisée par une kératite ulcéreuse unilatérale ou bilatérale rapidement progressive, intraitable et douloureuse, qui affecte initialement la périphérie de la cornée et progresse dans un premier temps autour du limbe puis de manière centripète. L'ulcération concerne uniquement le tissu cornéen stromal, laissant l'épithélium et l'endothélium intacts. La sclère adjacente n'est pas touchée. Les complications liées à la maladie sont un glaucome, une cataracte et une perforation."},"de":{"name":"Ulcus Mooren","synonyms":["Mooren-Ulkus"],"definition":"Eine seltene Erkrankung des vorderen Augenabschnittes, die durch eine rasch fortschreitende, persistierende, schmerzhafte, ulzerierende, ein- oder beidseitige Keratitis gekennzeichnet ist, die zunächst die periphere Hornhaut befällt und sich dann zirkulär und schließlich zentral ausbreiten kann. Der destruktive Prozess betrifft nur das Stroma der Hornhaut, Epithel und Endothel bleiben weitgehend intakt. Die benachbarte Sklera ist nicht betroffen. Die Erkrankung kann durch Glaukom, Katarakt und Perforation kompliziert werden."},"es":{"name":"Úlcera de Mooren","definition":"Es una entidad poco frecuente del segmento anterior del ojo caracterizada por queratitis ulcerosa uni- o bilateral rápidamente progresiva, intratable y dolorosa que afecta inicialmente a la córnea periférica y puede extenderse circunferencialmente y, con posterioridad, centralmente. El proceso destructivo afecta únicamente al tejido corneal del estroma respetando el epitelio y el endotelio en gran medida, sin afectación de la esclerótica adyacente. La afección puede complicarse con glaucoma, cataratas y perforación."},"it":{"name":"Ulcera di Mooren","definition":"Si tratta di una malattia rara del segmento anteriore dell'occhio, caratterizzata da una cheratite ulcerosa mono- o bilaterale, a progressione rapida, non trattabile e dolorosa, che inizialmente interessa la porzione periferica della cornea, per poi diffondersi verso l'esterno e successivamente, nella parte centrale. Il processo distruttivo coinvolge solo il tessuto corneale stromale, risparmiando l'epitelio e l'endotelio. La sclera non è interessata. Le complicanze comprendono il glaucoma, la cataratta e la perforazione."},"nl":{"name":"Ulcus van Mooren","definition":"Een zeldzame aandoening van voorste oogsegment, gekarakteriseerd door unilaterale of bilaterale, snel progressieve, refractaire, pijnlijke, ulceratieve keratitis die initieel perifeer hoornvlies treft en in de eerste plaats cirkelvormig en vervolgens centripetaal kan verspreiden. Het destructieve proces treft enkel stromaal weefsel van cornea, en laat epitheel en endotheel grotendeels ongemoeid. Er is geen betrokkenheid van aangrenzende sclera. De aandoening kan gecompliceerd worden door glaucoom, cataract, en perforatie."},"pt":{"name":"Úlcera Mooren"},"pl":{"name":"Wrzód Moorena"},"cs":{"name":"Moorenův vřed"},"uk":{"name":"Виразка Мурена","definition":"A rare disorder of the anterior segment of the eye characterized by a unilateral or bilateral rapidly progressive, intractable, painful, ulcerative keratitis which initially affects the peripheral cornea and may spread circumferentially and then centrally. The destructive process involves stromal corneal tissue only, leaving the epithelium and endothelium largely unaffected. There is no involvement of the adjacent sclera. The condition can be complicated by glaucoma, cataract, and perforation."},"zh":{"name":"蚕食性角膜溃疡"},"tr":{"name":"Mooren ülseri"}},"xrefs":[{"source":"ICD-10","code":"H16.0","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"9A76","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"470174149"},{"source":"MONDO","code":"0001032","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0155072","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97966,"name":"Rare ophthalmic disorder"},"parents":[{"orpha":519288,"name":"Rare disorder with corneal involvement as a major feature","classifications":[189]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":[],"prevalence":[],"phenotypes":null}