epithelial recurrent erosion dystrophy

Epithelial Recurrent Erosion Dystrophy (ERED) Description

Epithelial recurrent erosion dystrophy, also known as ERED, is a rare form of superficial corneal dystrophy characterized by frequent painful recurrent corneal erosions, with onset in the first decade of life [1][4]. This condition is marked by a disturbance at the level of the corneal epithelial basement membrane [8].

The symptoms of ERED include:

• Frequent and painful recurrent corneal erosions
• Onset in the first decade of life
• Subepithelial scarring and fibrosis, which may be mistaken for dystrophic deposits [3]

ERED is a treatable condition, but it requires proper management to prevent complications. The exact cause of ERED is not well understood, but it is believed to be related to defective adhesion between the corneal epithelium and the basement membrane [5].

Key Points:

• Frequent painful recurrent corneal erosions
• Onset in the first decade of life
• Subepithelial scarring and fibrosis
• Defective adhesion between the corneal epithelium and the basement membrane

References:

[1] Epithelial recurrent erosion dystrophy (ERED) is characterized by frequent painful recurrent corneal erosions, with onset in the first decade of life. [4] Epithelial recurrent erosion dystrophy (ERED) is characterized by frequent painful recurrent corneal erosions, with onset in the first decade of life. [3] Developing in childhood, the recurrent corneal erosions lead to subepithelial scarring and fibrosis, which may be mistaken for dystrophic deposits. By 30 to 40 ... [5] Recurrent corneal epithelial erosion syndrome is the result of defective adhesion to the basement membrane. The breakdown of corneal epithelium causes ...

Common Signs and Symptoms of Epithelial Recurrent Erosion Dystrophy (ERED)

Epithelial Recurrent Erosion Dystrophy (ERED) is a condition characterized by the spontaneous breakdown of the corneal epithelium, leading to recurring episodes of eye pain, redness, and vision disturbances. The following are common signs and symptoms associated with ERED:

• Watery tearing: Excessive tearing is a hallmark symptom of ERED, often accompanied by a burning sensation in the eyes [4].
• Sensitivity to light (photophobia): Patients with ERED may experience increased sensitivity to light, especially bright light [1], [3], [8].
• Blurry vision: Blurred vision can occur due to the corneal epithelial breakdown and subsequent inflammation [2], [5].
• Redness of the eye: Redness and inflammation of the eye are common symptoms of ERED [4], [6].
• Spasm of the eyelid (uncontrollable blinking): Some patients may experience uncontrollable blinking or spasms of the eyelid, particularly during episodes of pain [9].

Additional Symptoms

In addition to these primary symptoms, some patients with ERED may also experience:

• A burning sensation in the eyes
• Sensitive eyes for years
• Exposure keratopathy (exposure of the corneal surface due to incomplete closure of the eyelids)
• Recurrent episodes of acute pain, usually at night or upon first awakening

References

[1] Oct 11, 2024 — The spontaneous breakdown of the corneal epithelium can lead to the sudden onset of ocular pain, blurred vision, tearing, and photophobia, ...

[2] The condition, which may recur over weeks or months, can wake patients during the night, and cause lacrimation, photophobia and blurred vision.

[3] Most patients have attacks of redness, photophobia, epiphora, and ocular pain. Some experience a burning sensation and report sensitive eyes for years.

[4] Jan 30, 2024 — Signs & Symptoms · Lots of watery tearing · Sensitivity to light (especially bright light) · Blurry vision · Redness of the eye · Spasm of the ...

[5] An unpleasant sensation characterized by physical discomfort (such as pricking, throbbing, or aching) localized to the eye. ... Excessive sensitivity to light ...

[6] Mild to severe eye pain that typically develops during the night or upon awakening, The pain may be fleeting or can last for hours or days, depending on how ...

[7] Mar 20, 2023 — Recurrent corneal erosions and epithelial basement membrane dystrophy are usually bilateral and are characterized by various patterns of dots, ...

[8] RCES is characterised by repeated episodes of sudden onset of acute pain, usually at night or upon first awakening, accompanied by redness, photophobia and ...

[9] Jun 8, 2023 — Symptoms · sharp pains · sandy, gritty feeling in the eye · light sensitivity · tearing · redness · blurred vision · uncontrollable blinking ( ...

Diagnostic Tests for Epithelial Recurrent Erosion Dystrophy

Epithelial recurrent erosion dystrophy (ERED) can be diagnosed clinically, and genetic testing can be undertaken to confirm the diagnosis [5]. The diagnostic tests for ERED include:

• Slit lamp examination: A careful slit lamp examination often reveals corneal erosions [7]. Many times, there may be associated generalized or localized patches of corneal edema.
• Fluorescein staining: Special dyes such as fluorescein can be used to aid in the diagnosis [8].
• Genetic testing: Genetic testing can be undertaken using next-generation sequencing (NGS) methods to confirm the diagnosis, facilitate genetic counselling, provide accurate advice on prognosis and future family planning, and aid in clinical trial participation [5].

Other diagnostic tests

While not specifically mentioned as diagnostic tests for ERED, other tests that may be relevant include:

• Corneal topography: A noninvasive diagnostic test that measures the shape of the cornea, which can help diagnose associated conditions such as abnormal basement membrane dystrophy (ABMD) [15].
• In vivo confocal microscopy: This test demonstrates defects of the adhesion complex, deposits in basal epithelial cells, and sub-basal nerve plexus abnormalities in affected patients [3].

References

[1] OMIM - Epithelial recurrent erosion dystrophy [2] Clinical Management Guideline for Recurrent Corneal Erosions [3] In vivo confocal microscopy demonstrates defects of the adhesion complex, deposits in basal epithelial cells, sub-basal nerve plexus abnormalities in affected patients. [5] Diagnosis. Epithelial recurrent erosion dystrophy can be diagnosed clinically. Genetic testing can be undertaken to confirm the diagnosis, facilitate genetic counselling, provide accurate advice on prognosis and future family planning, and aid in clinical trial participation. [7] Mar 20, 2023 — A careful slit lamp examination often reveals corneal erosions. Many times, there may be associated generalized or localized patches of corneal edema. [8] TESTS AND DIAGNOSIS. The diagnosis can usually be made by history and slit lamp examination with special dyes such as fluorescein.

Treatment Options for Epithelial Recurrent Erosion Dystrophy (ERED)

Epithelial recurrent erosion dystrophy (ERED) is a rare form of superficial corneal dystrophy characterized by recurrent episodes of epithelial erosions from childhood in the absence of associated diseases, with occasional impairment of vision [15]. While there are no specific treatments for ERED, various management options can help alleviate symptoms and prevent complications.

Topical Lubrication Therapy

Most patients with recurrent corneal erosions respond to topical lubrication therapy, which involves applying artificial tears or ointments to the affected eye [4]. This treatment helps maintain a stable tear film, reducing discomfort and promoting healing.

Bandage Soft Contact Lenses

Bandage soft contact lenses can be used to protect the cornea during episodes of erosion. These lenses create a smooth surface for the epithelium to adhere to, facilitating healing and reducing pain [4].

Debridement of the Epithelium and Basement Membrane

In some cases, debridement (removal) of the affected epithelial layer may be necessary to promote healing and prevent further erosion. This procedure is typically performed by an eye care professional.

Pharmacological Treatment

Oral tetracyclines and topical steroid regimens have been used to inhibit matrix metalloproteinases, which can contribute to protein breakdown and corneal erosion [7]. Newer therapies include oral matrix metalloproteinase (MMP) inhibitors, blood-derived eye drops, amniotic membrane graft application, and others [9].

Other Management Options

In addition to these treatments, other management options may be considered on a case-by-case basis. These can include:

• Avoiding triggers such as sunlight, dust, or smoke
• Maintaining good sleep hygiene
• Using protective eyewear during activities that may exacerbate symptoms

It is essential to consult with an eye care professional for personalized guidance and treatment recommendations.

References: [4] [7] [9]

Understanding Differential Diagnosis in Epithelial Recurrent Erosion Dystrophy

Epithelial recurrent erosion dystrophy (ERED) is a condition characterized by recurring erosions on the surface of the cornea. When diagnosing ERED, it's essential to consider differential diagnoses that may present similar symptoms.

Conditions to Consider:

• Epithelial Basement Membrane Dystrophy: This condition also involves recurrent epithelial erosions and can be challenging to differentiate from ERED.
• Other Superficial or Stromal Corneal Dystrophies: Conditions like Meesman's Juvenile Epithelial Dystrophy or Reis-Bucklers' dystrophy may present with similar symptoms, making differential diagnosis crucial.

Key Differences:

• Location of Refractile Lines: In contrast to lattice dystrophy type I, the refractile lines in ERED are typically located peripherally and are fewer in number.
• Clinical Presentation: Patients with ERED often experience unilateral pain, lacrimation, photophobia, and blurred vision.

Importance of Accurate Diagnosis:

Accurate differential diagnosis is essential to ensure that patients receive the correct treatment for their condition. Misdiagnosis can lead to inappropriate treatment and potentially worsen symptoms.

References:

• [4] Suspected cases of ERED should be differentiated from other conditions that are accompanied by recurrent epithelial erosions, particularly when erosions are the initial presenting manifestation.
• [13] Corneal dystrophies must be differentiated among each other, as clinical presentations may often overlap.