{"orpha":98955,"name":"Lisch epithelial corneal dystrophy","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Band-shaped and whorled microcystic dystrophy of the corneal epithelium","LECD"],"definition":"Lisch epithelial corneal dystrophy (LECD) is a very rare form of superficial corneal dystrophy characterized by feather-shaped opacities and microcysts in the corneal epithelium arranged in a band-shaped and sometimes whorled pattern, occasionally with impaired vision.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-98955/","orphanet_url":"https://www.orpha.net/en/disease/detail/98955","names":{"fr":{"name":"Dystrophie cornéenne de Lisch","synonyms":["LECD"],"definition":"La dystrophie cornéenne de Lisch (LECD) est une forme très rare de dystrophie cornéenne superficielle caractérisée par des opacités en forme de plumes et des microkystes sur l'épithélium cornéen disposés en bandes et parfois en spirales, avec une vision parfois détériorée."},"de":{"name":"Lisch-epitheliale Hornhautdystrophie","definition":"Die Hornhautepithel-Dystrophie Lisch (LECD) ist eine sehr seltene Form von oberflächlicher Hornhautdystrophie und gekennzeichnet durch federförmige Trübungen und Mikrozysten im Hornhautepithel, die in einem band- und manchmal wirbelförmigen Muster angeordnet sind. Gelegentlich ist der Visus beeinträchtigt."},"es":{"name":"Distrofia corneal epitelial de Lisch","synonyms":["Distrofia microquística en forma de banda y verticilada del epitelio corneal","LECD"],"definition":"La distrofia corneal epithelial de Lisch (LECD) es una forma muy rara de distrofia corneal superficial caracterizada por opacidades en forma de pluma y microquistes en el epitelio corneal dispuestos en forma de banda y, en ocasiones, en espiral, con ocasionalmente problemas de visión."},"it":{"name":"Distrofia corneale epiteliale di Lisch","synonyms":["Distrofia microcistica a bande dell'epitelio corneale","LECD"],"definition":"La distrofia corneale epiteliale di Lisch (LECD) è una forma molto rara di distrofia superficiale della cornea, caratterizzata da opacità a forma di piuma e microcisti nell'epitelio corneale disposte a banda e, a volte, a spirale. Si associa raramente a deficit visivo."},"nl":{"name":"Epitheliale corneadystrofie van Lisch","synonyms":["Bandvormige en gedraaide microcystische dystrofie van hoornvliesepitheel","LECD"],"definition":"Epitheliale corneadystrofie van Lisch (LECD) is een zeer zeldzame vorm van oppervlakkige corneadystrofie gekenmerkt door veervormige opaciteiten en microcysten in het cornea-epitheel, die geordend zijn in een strookvormig en soms kransvormig patroon, af en toe met verstoord gezichtsvermogen."},"pt":{"name":"Distrofia corneana, tipo Lisch"},"pl":{"name":"Dystrofia nabłonkowa rogówki Lischa","synonyms":["LECD","Mikrotorbielowata dystrofia nabłonka rogówki o chrakterze smug i wirów"]},"cs":{"name":"Lischova epiteliální dystrofie rohovky","synonyms":["Pruhovitá a závitovitá mikrocystická dystrofie rohovkového epitelu","LECD"]},"uk":{"name":"Епітеліальна дистрофія рогівки Ліша","synonyms":["Стрічкоподібна та звивиста мікрокістозна дистрофія епітелію рогівки","LECD"],"definition":"Lisch epithelial corneal dystrophy (LECD) is a very rare form of superficial corneal dystrophy characterized by feather-shaped opacities and microcysts in the corneal epithelium arranged in a band-shaped and sometimes whorled pattern, occasionally with impaired vision."},"zh":{"name":"Lisch上皮细胞角膜营养不良","synonyms":["带状及螺旋状微囊性角膜上皮营养不良"],"definition":"LISCH上皮性角膜营养不良(LECD)是一种非常罕见的浅表性角膜营养不良，其特征是角膜上皮内有羽毛状混浊和微囊，呈带状，有时呈螺旋状排列，偶尔伴有视力障碍。"},"tr":{"name":"Lisch epitel kornea distrofisi","synonyms":["LECD","Kornea epitelinin bant şeklinde ve kıvrımlı mikrokistik distrofisi"]}},"xrefs":[{"source":"OMIM","code":"620763","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"H18.5","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"9A70.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1571503165"},{"source":"MONDO","code":"0010425","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"C567588","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C2749050","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97966,"name":"Rare ophthalmic disorder"},"parents":[{"orpha":522562,"name":"Genetic superficial corneal dystrophy","classifications":[156]},{"orpha":98625,"name":"Superficial corneal dystrophy","classifications":[189]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["X-linked dominant"],"onset":["Childhood"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":36.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}