Ulcerative Keratitis

Ulcerative keratitis is a serious inflammatory eye condition that causes painful sores and tissue breakdown in the cornea, requiring immediate medical attention to prevent vision loss and, in some cases, life-threatening complications.

Table of contents

• What Is Ulcerative Keratitis
• Types of the Condition
• What Causes This Condition
• Signs and Symptoms
• How Doctors Diagnose the Condition
• Treatment Options
• Outlook and Long-Term Effects

What Is Ulcerative Keratitis

Ulcerative keratitis is a condition that affects the cornea, which is the clear, dome-shaped layer covering the front of your eye, protecting the colored part (iris) and the pupil. When this clear covering becomes inflamed and develops open sores or ulcers, it can cause significant pain and vision problems.^[1]

The term “keratitis” simply means inflammation of the cornea, and when ulcers form, doctors describe it as ulcerative keratitis. This condition is considered a medical emergency because it can lead to permanent eye damage, severe vision loss, or even blindness if not treated quickly.^[3]

Types of the Condition

There is a specific form of ulcerative keratitis called peripheral ulcerative keratitis (also known as marginal keratolysis or peripheral rheumatoid ulceration). This type affects the edge or margin of the cornea, near where it meets the white part of the eye. The condition often appears as a crescent-shaped area of tissue breakdown and thinning in this outer zone of the cornea.^[1][2]

Peripheral Ulcerative Keratitis, Marginal Keratolysis, Peripheral Rheumatoid Ulceration

Peripheral ulcerative keratitis is relatively rare, affecting an estimated 0.2 to 3 people per million each year.^[12] This form of the condition is particularly serious because it often signals the presence of other health problems throughout the body.

One specific type of peripheral ulcerative keratitis that occurs without any related body-wide disease is called Mooren ulcer. This represents about 31.5 percent of all peripheral ulcerative keratitis cases and is diagnosed only after doctors have ruled out other possible causes.^[2]

What Causes This Condition

Peripheral ulcerative keratitis develops through a complex process involving the body’s immune system, the unique structure of the outer cornea, and various triggering factors. The condition can have either infectious or non-infectious causes.^[2]

The most common cause involves an autoimmune reaction, where the body’s defense system mistakenly attacks its own corneal tissue. In this process, antibodies or immune cells that normally protect against infections instead target the body’s own tissues, causing inflammation and damage.^[1]

Many people with peripheral ulcerative keratitis have underlying systemic rheumatic disorders—conditions that affect connective tissue throughout the body. Connective tissue is the structural material that gives strength to joints, tendons, ligaments, and blood vessels. The rheumatic conditions most commonly associated with this eye problem include:^[1][2]

• Rheumatoid arthritis (accounting for about 34 percent of non-infectious cases)
• Granulomatosis with polyangiitis
• Relapsing polychondritis
• Systemic lupus erythematosus
• Polyarteritis nodosa

Together, these autoimmune diseases can account for more than half of all peripheral ulcerative keratitis cases.^[2][12]

Infectious causes can also lead to peripheral ulcerative keratitis. These include bacteria, fungi, viruses like herpes simplex, and parasites.^[1][4] Infections are responsible for approximately 19.7 percent of peripheral ulcerative keratitis cases.^[12]

The peripheral cornea is particularly vulnerable to inflammation because, unlike the central cornea, it has direct blood supply from tiny vessels extending from the edge of the cornea. This blood supply can bring inflammatory substances directly to the area. The outer cornea also has access to the body’s lymphatic system, which allows immune system cells to reach this region more easily.^[6][12]

Signs and Symptoms

People with peripheral ulcerative keratitis typically experience several uncomfortable symptoms. The most common include:^[1][4][12]

• Blurred or hazy vision
• Increased sensitivity to bright light (a condition called photophobia)
• A sensation that something is stuck in the eye, like a hair or piece of dust
• Eye pain, which can range from mild discomfort to severe
• Redness of the eye
• Excessive tearing

When doctors examine the eye, they can see the ulcer located at the margin or edge of the cornea. It usually appears oval or crescent-shaped.^[1] In severe cases, the progressive breakdown of corneal tissue can lead to extreme thinning that may result in a bulging area or even a hole (perforation) in the cornea, which is a medical emergency.^[2]

About one-third of people with peripheral ulcerative keratitis also develop scleritis, which is inflammation of the white outer layer of the eye. When scleritis is present along with the corneal ulcer, the condition tends to be more serious.^[2][14]

How Doctors Diagnose the Condition

Peripheral ulcerative keratitis is primarily a clinical diagnosis, meaning doctors identify it based on what they see during examination rather than through laboratory tests alone. However, a thorough evaluation is essential because this eye condition can be the first sign of a serious, potentially life-threatening disease affecting the whole body.^[2][14]

The doctor will examine your eye using a special instrument called a slit lamp, which provides bright light and magnification. This allows them to see the characteristic crescent-shaped damage and thinning in the peripheral cornea, along with the overlying tissue defect.^[7][14]

Doctors suspect peripheral ulcerative keratitis when they observe an affected cornea in someone who also has a severe or long-standing systemic rheumatic disease.^[1] However, because many conditions can look similar, doctors must rule out other possibilities through careful evaluation.

If doctors suspect an infection is causing the problem, they will scrape the ulcer and the edges of the eyelids to collect a sample. This sample is then grown in a laboratory (a process called culturing) to identify the specific organism causing the infection, whether it’s bacteria, fungi, or a virus.^[1][4]

A comprehensive workup typically includes laboratory testing to look for autoimmune, rheumatic, and infectious causes. Common tests include:^[12][14]

• Rheumatoid factor (RF) to check for rheumatoid arthritis
• Inflammatory markers including erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)
• Antinuclear antibody (ANA) test for connective tissue diseases
• ANCA (antineutrophil cytoplasmic antibody) screening for certain types of blood vessel inflammation
• Tests for tuberculosis infection
• Complete blood count and metabolic panel
• Urinalysis
• Chest X-ray

Because peripheral ulcerative keratitis often requires coordination with other medical specialists, patients may also be referred to a rheumatologist (a doctor who specializes in autoimmune and joint diseases) or an internist for further evaluation.^[13][14]

Treatment Options

Treating peripheral ulcerative keratitis requires prompt and aggressive action to prevent corneal perforation and address any underlying disease. Treatment approaches vary depending on whether an infection is present and whether there is an associated systemic condition.^[2]

For the local eye problem, doctors typically prescribe medications that suppress the immune system. These may include methotrexate, cyclophosphamide, rituximab, or etanercept, which can be taken by mouth or given through a vein (intravenously).^[1][4] Recent studies have shown that rituximab has a particularly high success rate, achieving steroid-free remission in about 63 percent of cases.^[13]

Treatment often begins with systemic steroids to control local inflammation. Doctors may start with intravenous methylprednisolone or oral prednisone. However, because long-term steroid use has significant side effects, many patients will eventually transition to steroid-sparing medications for continued control of the condition.^[13][14]

Topical treatments applied directly to the eye include:^[2][13]

• Corticosteroid eye drops to reduce inflammation (though these must be used carefully as they can promote further corneal thinning)
• Prophylactic antibiotic drops to prevent bacterial infection
• Immunosuppressive agents like cyclosporine eye drops
• Artificial tears

Some doctors also prescribe oral doxycycline and vitamin C, as these can help prevent corneal thinning by blocking certain destructive enzymes and promoting the formation of new structural proteins.^[14]

When the cornea has become dangerously thin or the ulcer is not healing with medication alone, surgical intervention may be necessary. Options include:^[1][4]

• Filling the ulcer with a special tissue adhesive and covering it with a bandage contact lens
• Placing an amniotic membrane graft over the affected area
• Performing a partial thickness corneal transplant (patch graft)
• Removing inflamed tissue near the ulcer

Close monitoring is essential during treatment. Initially, patients may need to see their doctor daily to ensure the eye is healing properly.^[15] Coordination with rheumatology specialists is often necessary for managing any underlying systemic disease.^[14]

Outlook and Long-Term Effects

The outlook for people with peripheral ulcerative keratitis depends significantly on whether they have an underlying systemic rheumatic disease and how quickly treatment begins.

Without treatment, the prognosis is quite serious. Among people who have a systemic rheumatic disease along with peripheral ulcerative keratitis, approximately 40 percent die within 10 years of developing the eye condition, most commonly from heart attack. However, it’s important to understand that the eye problem itself is not the cause of death—rather, it’s the effects of the underlying rheumatic disorder on the whole body.^[1][4]

The good news is that treatment dramatically improves survival. With proper systemic immunosuppressive therapy, the 10-year mortality rate drops to about 8 percent.^[1][7] This represents a significant improvement and underscores the importance of early diagnosis and aggressive treatment.

The condition itself poses a direct threat to vision. Progressive breakdown of corneal tissue can lead to corneal perforation, which is a medical emergency that can result in permanent vision loss or blindness.^[2] When peripheral ulcerative keratitis occurs alongside scleritis (inflammation of the white of the eye), the prognosis tends to be worse.^[2]

Because peripheral ulcerative keratitis can be the first presenting sign of a life-threatening systemic disease, quick recognition and comprehensive evaluation are crucial. The condition requires ongoing management and regular follow-up care to monitor both the eye condition and any associated systemic illness.^[2][14]