Wegener肉芽肿双侧穿孔性巩膜软化及周边角膜变薄VIP免费

IntJOphthalmol，V01．9，No．7，Jul．2009w删．UO．cnTel：029-8224517283085628Email：IJO．2000@163．com·Casereport·Bilateralscleromalaciaperforansandperipheralcorneal-’‘‘●IT7■’·‘thinninglnWegener7sgranulomatosisscReddy，ITajunisah，TRohanaDepartmentofOphthalmology，FacultyofMedicine，UniversityofMalaya，KualaLumpur，MalaysiaCorrespondenceto：SCReddy．DepartmentofOphthalmology，UCSISchoolofMedicine，BukitKor，MukimRusila，21600Marang，Terengganu，Malaysia．profscreddy@gmail．cornReceived：2009．04-20Accepted：2009—06-22Abstract·ArarecaseofbilateraIscleromalaciaperforans．bilateraIperipheralcornealthinning(contactlenscornea)andunilateraIorbitaIinflammatorydiseaseina50yearoldfemalepatientwithanindolentformWegener’sgranulomatosis(WG)involvinglungsandsinusesisreported．Thepatientsurvivedfor12yearsaftertheinitiaIdiagnosisofsystemicdisease．TherewasperforationofleftglobefollowingtraumaandnoperforationoftherightglobetilltheIastfoIlowupofthepatient．·KEY＼／VORDS：WegeneYsgranulomatosis；scleromalaciaperforans；peripheralthinningofcornea；proptosisDOI：10．3969／1．issn．1672-5123．2009．07．007ReddySC，TajunisahI，RohanaT．BilateralscleromalaciapefforansandperipheralcornealthinninginWegenefsgranulomatosis．IntJOphthalmol(aHojiYnnkeZnzhi)2009；9(7)：1239-1242INTRoDUCTloNw盖淼=：：=舒：墨二：：da冒伦：盅=inflammationofupperandlowerrespiratorytract，necrotizingvasculitisandnephritis．Thediseaseexistsintwoforms；anindolentform(1imited，local—regional，withabsenceofrenalinvolvement)andfulminateform(active，generalized)．Ocularinvolvementcanoccurinbothformsofthedisease，withareportedincidencerangingfrom28％to60％ofcasesandashishas87％atsometimeduringthepatient’slife．Itmaybepresentatthetimeofdiagnosisorinfactmaybethepresentingfeaturein8％to16％ofpatients⋯¨．ThemostoomnlonocularlesionsobservedinWGpatients撇orbitalinflammatorydisease，necrotizingscleritisandperipheralulcerativekeratitis．Orbitalinvolvementismanifestedasproptosisandisduetoprimarygranulomatousinflammationorcontiguousspreadofthediseasefromadjacentparanasalsinuses．Peripheralulcerativekeratitisisadestructiveinflammationofthecornealperipheryassociatedwithailepithelialdefectproducingulcerationandperipheralcornealthinning．Itiscommonlyaccompaniedbyscleritisofthenecrotizingtypegivingrisetoredpainfuleyeandblurringofvision．Occasionally，nonulcerativetypeofperipheralkeratitismaymanifestasperipheralstromalthinningcharacterizedbygradualresorptionofperipheralstromaleavingtheepitheliumintact．Endstageofthismayresultinacontactlenscorneaappearance．ThisisseeninmorecommonlyinrheumatoidarthritisthaninWG．Scleromalaciapefforansisatypeofanteriornecrotizingscleritiswithoutinflammationresultinginprogressivescleralthinning。⋯．Pubmedseachrevealedonlyonecaseofbilateralscleromalaciaperforans‘10]，andthreecasesofperipheralcornealthinningL11,12JinWGpatients．Wedescribeararecaseofbilateralseleromalaciaperforans，bilateralperipheralcornealthinningandunilateralorbitalinflammatorydiseaseinapatientwith锄indolentformWGinvolvinglungsandsinuses．CASEREPORTA50yearoldChineseladywasfirstdiagnosedtohaveWGinAugust1992whenshepresentedwithcough，hemoptysis，feverandweightloss．Shehadnasalobstruction，foulsmellingnasaldischarge，rhinitis，bilateralmaxillarysinusitis．Therewassclerathinningandperipheralcornealthinningintheuppernasalquadrantinth...