Behçet’s Disease: A Case of Peripheral Ulcerative Keratitis Leading to Corneal Perforation Selcuk Sizmaz, Aysel Pelit, Meltem Yagmur, Didem Arslan, Yonca Aydin Akova

A 27 year-old man Two day history of redness in the right eye Corneal thinning involving the anterior stroma and adjacent conjunctival hyperemia, in the corneal limbus, at the 2- to 3-o’clock quadrant No sign of dry eye Topical ofloxacin and artificial tears prescribed

Three days later, signs worsened –Thinning involved the posterior stroma –Patient declined surgery

Two days later –Corneal perforation –İris prolapsed

Corneal patch graft and amniotic membrane transplantation

Ophthalmic and systemic evaluation No significant ocular history Oral aphthous lesions that had occurred once or twice a month for the previous 2 years Eight years history of lower back and buttock pain with morning stiffness for 30 minutes Arthralgia in the knee joints without swelling

Arterial BP (mmHg) –130/80 right arm –100/70 left arm Weak pulse in left arm Colour Doppler USG: left subclavian artery narrowing MR angipgraphy: 4 cm- long diffuse stenosis in left subclavian artery Sacroiliac graphy: grade II-III sacroiliitis Pathergy test: (-) Erithrocyte sedimantation rate 35 mm/h C-reactive protein4.5 mg/L (↑) HBV(-) HCV(-) HIV(-) ANA(-) C-ANCA(-) HLA-B27(-) HLA-B51(-)

Diagnosis: Behçet’s disease Oral aphthous lesions Ocular involvement Large vessel vasculitis Sacroiliitis

Management: –Methylprednisolone, 32 mg/day, po; tapered –Azathioprine, 150 mg/day, po Prognosis (1 year follow-up): –No recurrence –Systemic symptoms improved

Differential diagnosis Takayasu arteritis +Aorta and branches involved –No aphthous lesions –Sacroiliitis uncommon –Ocular invovement rare Polyarteritis nodosa +Peripheral ulcerative keratitis occurs –Predominantly females –Aneurisms in small-middle sized vessels –Constitutional symptoms