(See also Introduction to Corneal Disorders.)
Peripheral ulcerative keratitis is a serious corneal ulceration; it often occurs with autoimmune connective tissue diseases that are active, long-standing, or both, such as rheumatoid arthritis (RA), granulomatosis with polyangiitis (formerly called Wegener granulomatosis), and relapsing polychondritis.
Patients often have decreased visual acuity, photophobia, and foreign body sensation. A crescentic area of opacification develops in the periphery of the cornea due to infiltration by white blood cells (WBCs). Ulceration, which stains with fluorescein, develops shortly after opacification. This is the opposite to the usual infectious ulcer that begins with an epithelial defect and later develops opacification. Infectious causes, such as bacteria, fungi, and herpes simplex virus, must be ruled out by culturing the ulcer and eyelid margins.
Among patients with autoimmune connective tissue disease and peripheral ulcerative keratitis, the 10-year mortality rate is about 40% (usually due to myocardial infarction) without treatment and about 8% with systemic cytotoxic therapy.
Any patient with peripheral ulcerative keratitis should be promptly referred to an ophthalmologist. Systemic cyclophosphamide, other immunosuppressants, or biologicals such as rituximab treat the keratitis, life-threatening vasculitis, and underlying autoimmune disease. Treatment also includes local approaches to control inflammation (eg, tissue adhesive and bandage contact lenses) and repair damage (eg, patch grafts). Other possibly helpful drugs include collagenase inhibitors, such as systemic tetracycline or topical 20% N-acetylcysteine.