Mooren's ulcer

Mooren's Ulcer Description

Mooren's ulcer is a rare idiopathic ocular disorder that may lead to blindness due to progressive destruction of the peripheral cornea [1]. It is characterized by a markedly painful ulcerative keratitis that can be rapidly progressive, relentless, and resistant to therapeutic intervention [2][7].

The disease typically begins with intense limbal inflammation and swelling in the episclera and conjunctiva [11]. Corneal changes begin within 2-3 mm from the limbus, first appearing as grey swellings that rapidly furrow, affecting the superficial one-third of the cornea and then progressing to more central areas [11].

Mooren's ulcer is often associated with extensive corneal vascularization and scarring [5]. It can be bilateral in approximately one-third of cases and the cause is unknown [6].

Key Features:

• Markedly painful ulcerative keratitis
• Rapidly progressive and relentless
• Resistant to therapeutic intervention
• Intense limbal inflammation and swelling
• Corneal changes begin within 2-3 mm from the limbus
• Extensive corneal vascularization and scarring

References:

[1] Context result 1 [2] Context result 2 [5] Context result 5 [6] Context result 6 [7] Context result 7 [11] Context result 11

Common Signs and Symptoms of Mooren's Ulcer

Mooren's ulcer is a rare idiopathic ocular disorder that can lead to blindness due to progressive destruction of the peripheral cornea. The symptoms of Mooren's ulcer can include:

• Pain in the affected eye(s): Patients often report severe pain, which can be out of proportion to the clinical signs [7].
• Redness of the affected eye(s): Redness and inflammation are common symptoms of Mooren's ulcer.
• Progressive ulceration of the cornea: The ulcer starts in the periphery of the cornea and spreads both centripetally and towards the sclera, leading to extensive corneal vascularization and scarring [5].
• Severe pain, photophobia, lacrimation, and defective vision: These symptoms are often reported by patients with Mooren's ulcer [11].

It is essential to note that Mooren's ulcer can be confused with other conditions, such as corneal ulcers caused by systemic autoimmune disorders. Therefore, a diagnosis of exclusion is necessary.

References:

[1] Context 1: Mooren's ulcer is a rare idiopathic ocular disorder... [5] Context 5: Mooren's ulcer is a typically progressive and painful ulceration... [7] Context 7: In Mooren ulcers, the pain is often out of proportion to the clinical signs. [11] Context 11: Mooren’s ulcer symptoms. These include severe pain...

Diagnostic Tests for Mooren's Ulcer

Mooren's ulcer is a diagnosis of exclusion, which means that it can only be confirmed after ruling out other possible causes of peripheral corneal ulceration. The following diagnostic tests are typically used to diagnose and rule out other conditions:

• Complete Blood Count (CBC): A CBC test measures the levels of different blood cells, including red and white blood cells, platelets, and hemoglobin. This test can help identify any underlying systemic diseases that may be contributing to the Mooren's ulcer.
• Urinalysis (UA), Blood Urea Nitrogen (BUN), and Creatinine: These tests measure the levels of waste products in the urine and blood, which can indicate kidney function or other systemic issues.
• Erythrocyte Sedimentation Rate (ESR): ESR measures how quickly red blood cells settle to the bottom of a test tube containing a blood sample. A high ESR level can indicate inflammation or infection.
• C-Reactive Protein (CRP): CRP is a protein that is produced by the liver in response to inflammation. Elevated levels of CRP can indicate an underlying inflammatory condition.
• Rheumatoid Factor: This test measures the presence of rheumatoid factor, an antibody that can be associated with autoimmune diseases such as rheumatoid arthritis.
• Antinuclear Antibodies (ANA): ANA tests measure the presence of antibodies against the nucleus of cells. A positive result can indicate an underlying autoimmune disease.
• Anti-Neutrophil Cytoplasmic Antibodies (ANCA): ANCA tests measure the presence of antibodies against neutrophils, a type of white blood cell. A positive result can indicate vasculitis or other inflammatory conditions.
• Complement Fixation: This test measures the levels of complement proteins in the blood, which are involved in the immune response.
• Corneal Scrapings with Bacterial, Fungal, and Viral Cultures: If infection is suspected, corneal scrapings can be taken for culture to identify the causative pathogen.

It's worth noting that Mooren's ulcer is a diagnosis of exclusion, and these tests are used to rule out other possible causes of peripheral corneal ulceration. A thorough medical history and review of systems should also be performed to determine if there are any underlying systemic diseases that may be contributing to the condition.

References:

• [3] Testing/Laboratory work-up.
• [4] Apr 6, 2023 — Laboratory Studies · Complete blood cell (CBC) count · Urinalysis (UA), blood urea nitrogen (BUN), and creatinine · Erythrocyte sedimentation rate
• [10] ANCA testing can help diagnose vasculitides like GPA. Microbiological testing: If infection is suspected, corneal scrapings with bacterial, fungal, and viral cultures can help identify the pathogen involved.
• [11] In Mooren's Ulcer (which by definition is of unknown etiology and not associated with systemic autoimmune disease), unregulated helper Th1 cells have been shown to produce autoreactive antibodies.

Medical Management of Mooren's Ulcer

Mooren's ulcer can be effectively managed through medical treatment, which includes the use of various drugs to control inflammation and promote healing.

• Steroids: Topical corticosteroids are often used as a first-line treatment for Mooren's ulcer. They help reduce inflammation and alleviate symptoms [1, 3, 9].
• Non-steroidal anti-inflammatory drugs (NSAIDs): These medications can also be used to manage pain and inflammation associated with Mooren's ulcer [1, 3].
• Cyclosporine: Topical cyclosporine has been suggested as a treatment option for Mooren's ulcer, particularly in cases where corticosteroids are not effective [9].
• Immunomodulators: Systemic immunomodulators such as rituximab and infliximab have been used to treat severe and refractory Mooren's ulcers [5, 7].

Systemic Approach

In some cases, a systemic approach may be necessary to manage progressive Mooren's ulcer. This can include the use of immunosuppressive therapy to control inflammation and promote healing [8].

It is essential to note that there is no evidence to suggest which treatment modality is the most effective among these options. A comprehensive treatment plan should be tailored to individual patient needs, taking into account the severity and progression of the disease.

References: [1] - Context 2 [3] - Context 3 [5] - Context 5 [7] - Context 7 [8] - Context 8 [9] - Context 9

Differential Diagnosis of Mooren's Ulcer

Mooren's ulcer, also known as peripheral ulcerative keratitis (PUK), requires a thorough differential diagnosis to rule out other painful peripheral ulcerations and systemic diseases. The following conditions must be considered in the differential diagnosis:

• Infectious Keratitis: This is an infection of the cornea that can cause pain and ulcers.
• Peripheral Ulcerative Keratitis (PUK): A type of corneal inflammation that can lead to ulceration.
• Systemic Malignancies: Certain types of cancer, such as lymphoma or leukemia, can cause Mooren's ulcer.
• Vasculitis: Inflammation of the blood vessels, which can lead to Mooren's ulcer.
• Collagen Diseases: Conditions like Wegener granulomatosis, polyarteritis nodosa, and rheumatoid arthritis (RA) can be associated with Mooren's ulcer.
• Rheumatoid Furrow: A type of corneal inflammation that can cause ulcers and pain.

According to [3], Mooren's ulcer is likely an autoimmune disorder that is often idiopathic but can be associated with systemic disease, including herpes simplex and zoster. It must also be differentiated from other painful peripheral ulcerations, as stated in [4].

The differential diagnosis for Mooren's ulcer is extensive, and a careful review of the system is necessary to rule out these conditions. As mentioned in [5], the differential diagnosis for Mooren's ulcer is similar to that for PUK.

In addition to these conditions, Mooren's ulcer can also be associated with connective tissue diseases, such as rheumatoid arthritis (RA), Sjögren syndrome, and others, as stated in [7].

References:

[3] Nov 12, 2001 - Mooren's ulcer is likely an autoimmune disorder that is often idiopathic. But it can be associated with systemic disease including herpes simplex and zoster.

[4] Jan 20, 2022 - It must be differentiated from other painful peripheral ulcerations including infectious keratitis and peripheral ulcerative keratitis (PUK).

[5] by QD Nguyen - The differential diagnosis for Mooren's ulcer is that for peripheral ulcerative keratitis, and can be quite extensive.

[7] Apr 5, 2023 - This type of corneal ulcer is usually associated with a connective tissue disease, such as rheumatoid arthritis (RA), Sjögren syndrome.