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...