Case Presentation: A 50 year-old gentleman with seronegative rheumatoid arthritis on leflunamide presented with foreign body sensation, irritation, pain and photophobia in bilateral eyes which acutely worsened to bilateral vision loss. Funduscopic and slit-lamp examination showed crusting, moderate erythema, and edema of both eyes. Further detailed exam showed mucoid discharge, bilateral corneal infiltrates, corneal neovascularization, diffuse corneal haze of right eye, and limbal thinning of left eye. Also, visual acuity was significantly decreased in both eyes. The patient’s history of rheumatoid arthritis and the exam findings of corneal infiltrates with neovascularization and limbal thinning established the diagnosis of acute and rapid bilateral corneal melting. Cyclophosphamide and intravenous steroids were initiated. Empiric oral and ophthalmic antibiotics were started for possible infectious etiology, which were then titrated off once infection was ruled out. Serologic workup for other autoimmune etiologies was negative. Patient’s vision improved after two days of therapy, however, on day seven, he had a left corneal perforation and subsequently underwent corneal transplantation. His vision improved after surgery, and he was discharged on ophthalmic steroids and a long course of oral prednisone.
Discussion: Corneal melt is a rare but serious complication of rheumatoid arthritis which usually manifests between 12 and 35 years after the initial diagnosis. It is characterized by inflammation involving the limbal part of the cornea and sclera, collagen destruction, cellular infiltration and limbal vascular changes indicative of vasculitis. Although Herpes simplex virus keratitis and retained lenticular material are the two most common causes of corneal melt, other etiologies include Sjogen’s, SLE, Granulomatosis with polyangiitis, as well as complications due to intraocular surgeries, such as cataract extraction.
Corneal melt symptoms are characterized by severe pain, photophobia, sensation of foreign bodies, and excessive watering. Serious eye conditions that can be sight-threatening, such as corneal melt, exhibit red-flag features that should not be missed, which include moderate-severe pain, photophobia, reduced visual acuity, eye trauma, and unilateral marked redness. These red-flags are associated with keratitis, scleritis, acute glaucoma, foreign body trauma, chemical burns, and orbital cellulitis. These entities should be part of the differential diagnoses when evaluating a red eye with red-flag features. Diagnosis is confirmed with slit lamp examination, which is necessary to narrow the differential diagnosis of the red eye. Infectious etiologies must also be excluded. Treatment must be early and aggressive due to a high mortality rate. Cyclophosphamide is the treatment of choice in conjunction with steroids, and the usual surgical treatment is scleral or corneal grafting. Duration of treatment is patient-specific based on presence of underlying systemic vasculitis, response to medical treatment, need for surgical intervention, and presence of concomitant infections. Corneal perforation is a feared complication of corneal melt and is typically treated with corneal transplantation.
Conclusions: Certain red eye diseases exhibit red-flag signs and symptoms which should not be missed as these diseases are not only sight-threatening but also life-threatening.