Behçet’s Disease: A Case of Behçet’s Disease: A Case of Peripheral Ulcerative Keratitis Peripheral Ulcerative Keratitis Leading to Corneal PerforationLeading 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 Ophthalmic and systemic evaluationevaluation

• 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 protein 4.5 mg/L (↑)

HBV (-)

HCV (-)

HIV (-)

ANA (-)

C-ANCA (-)

HLA-B27 (-)

HLA-B51 (-)

Diagnosis: Behçet’s diseaseDiagnosis: 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 diagnosisDifferential 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