Symptoms include blurred vision, increased sensitivity to bright light, and a sensation of a foreign object in the eye.
Doctors diagnose peripheral ulcerative keratitis based on the appearance of the cornea in someone who has a connective tissue disorder.
Doctors treat the damaged cornea and give drugs to suppress the immune system.
(See also Introduction to Corneal Disorders Introduction to Corneal Disorders The cornea is the clear layer in front of the iris and pupil. It protects the iris and lens and helps focus light on the retina. It is composed of cells, protein, and fluid. The cornea looks... read more .)
Peripheral ulcerative keratitis is probably caused by an autoimmune reaction. In an autoimmune disorder Autoimmune Disorders An autoimmune disorder is a malfunction of the body's immune system that causes the body to attack its own tissues. What triggers autoimmune disorders is not known. Symptoms vary depending on... read more , antibodies or cells produced by the body attack the body’s own tissues. Many autoimmune disorders affect connective tissue in a variety of organs. Connective tissue is the structural tissue that gives strength to joints, tendons, ligaments, and blood vessels. The connective tissue disorders that occur with peripheral ulcerative keratitis include rheumatoid arthritis Rheumatoid Arthritis (RA) Rheumatoid arthritis is an inflammatory arthritis in which joints, usually including those of the hands and feet, are inflamed, resulting in swelling, pain, and often destruction of joints.... read more , granulomatosis with polyangiitis Granulomatosis with Polyangiitis Granulomatosis with polyangiitis often begins with inflammation of small- and medium-sized blood vessels and tissues in the nose, sinuses, throat, lungs, or kidneys. The cause is unknown. The... read more , and relapsing polychondritis Relapsing Polychondritis Relapsing polychondritis is a rare autoimmune rheumatic disorder characterized by episodes of painful, destructive inflammation of the cartilage and other connective tissues in many organs.... read more .
Peripheral ulcerative keratitis also may be caused by an infection.
The diagnosis of peripheral ulcerative keratitis is suspected when the doctor sees an affected cornea in a person who also has a severe and/or long-standing connective tissue disease.
If doctors suspect an infection, such as bacteria, fungi, or herpes simplex virus, is the cause of peripheral ulcerative keratitis, they scrape the ulcer and eyelid margins for a sample. The sample is then grown in a laboratory (cultured) to identify the organism.
Without treatment, about 40% of people who have an autoimmune connective tissue disease and peripheral ulcerative keratitis die (mostly due to a heart attack) within 10 years of developing peripheral ulcerative keratitis. With treatment, about 8% of people die within 10 years. The eye problem itself is not the cause of death; it is the underlying autoimmune disorder's effect on the whole body.
To treat peripheral ulcerative keratitis, doctors give drugs that suppress the immune system, such as methotrexate, cyclophosphamide, or rituximab taken by mouth or by vein (intravenously).
Doctors may also fill the ulcer with a special material (called tissue adhesive) and then cover the area with special contact lenses to control inflammation. Alternatively, doctors may surgically repair the cornea with a partial thickness corneal transplant Corneal Transplantation Corneal transplantation (keratoplasty) is a common and highly successful type of transplantation. A scarred, severely painful, perforated, misshapen, or cloudy cornea (the clear layer in front... read more .