Peripheral ulcerative keratitis in Behçet’s disease: A rare bilateral presentation with subsequent corneal perforation

Abstract

Peripheral Ulcerative Keratitis (PUK) is marked by stromal inflammation and degradation of the cornea and is a rare ocular presentation  of Behçet’s Disease (BD). Early diagnosis and treatment are essential to prevent ocular morbidity. A 36-year-old male known to have BD presented with one month duration of bilateral ocular pain, redness, tearing, photophobia and impaired vision. There was previous  history of oral and genital ulcers, skin rash, arthritis and subacute deep venous thrombosis of the superior vena cava. Slit lamp revealed a  right inferotemporal corneal ulcer (3x1mm) with a vascularized margin and a left inferonasal corneal perforation with lost anterior  chamber and iris prolapse; with no evidence of intraocular inflammation. The unaided visual acuity was 0.05 (right) and 0.016 (left).  Scleral patch graft was used to repair the left corneal perforation. Autoimmune profile and thrombophilia screen were negative. The  patient was treated with pulse methylprednisolone (1g/day) for 3 days followed by cyclophosphamide (1g/month) for 6 months; then he  was maintained on oral steroids, azathioprine (150mg/day), oral colchicine (1mg/day) and anticoagulation. After one month, the ocular  pain decreased and hyperemia resolved with improved vision. On follow-up, slit-lamp revealed right clear cornea and left healed scleral patch (2x3mm) at 8.30 o’clock. BD may atypically present with bilateral PUK; which might be complicated by corneal perforation. This case  contributes to raise the awareness of rheumatologists and ophthalmologists regarding this unusual presentation of BD. Patients  with PUK may require evaluation for coexistent rheumatic diseases.