epithelial basement membrane dystrophy

ICD-10 Codes

Related ICD-10:

Description

Epithelial Basement Membrane Dystrophy (EBMD): A Common Corneal Condition

Epithelial basement membrane dystrophy, also known as anterior basement membrane disease or map-dot-fingerprint dystrophy, is a common condition that affects the cornea of the eye. It is characterized by an abnormal thickening of the epithelial basement membrane, leading to various symptoms and complications.

Key Features:

  • Bilateral condition: EBMD typically affects both eyes, although the severity may vary between them.
  • Age range: The condition usually develops between the ages of 20 and 50 years.
  • Subepithelial microcysts: Histologically, there is often an irregular, thickened epithelial basement membrane with subepithelial microcysts.
  • Fingerprint lines and geographic maplike lines: The cornea may exhibit fingerprint-like lines or geographic map-like patterns.

Symptoms:

  • Fluctuating vision: Patients may experience fluctuating vision, which can range from mild to severe.
  • Poor vision: EBMD can cause poor vision, which may be temporary or persistent.
  • Sensitivity to light: Some individuals may feel sensitive to light or experience pain in the affected eye.
  • Redness and irritation: Redness, irritation, and a foreign body sensation are also common symptoms.

Causes:

  • Genetic mutations: EBMD is caused by genetic mutations, which can be hereditary or occur randomly during cell division.
  • Environmental factors: Environmental factors such as UV radiation may contribute to the development of EBMD.

Complications:

  • Scarring from recurrent corneal erosions: Recurrent corneal erosions can lead to scarring and loss of best corrected visual acuity.
  • Decreased vision: EBMD can cause decreased vision, which may be permanent or temporary.

The information provided in this response is based on the following search results:

[1] Epithelial Basement Membrane Dystrophy (EBMD), is the most common of the corneal dystrophies. [2] It is also known as Map-Dot-Fingerprint Dystrophy and Cogan microcystic epithelial dystrophy. [3] EBMD, also known as map-dot-fingerprint dystrophy and Cogan microcystic epithelial dystrophy, is a corneal epithelial disease that may result in recurrent corneal erosions, irregular corneal astigmatism, and decreased vision.

[4] Epithelial basement membrane dystrophy (EBMD) is a disorder of the eye that can cause pain and dryness. [5] EBMD, also known as map-dot-fingerprint dystrophy and Cogan microcystic epithelial dystrophy, is a corneal epithelial disease that may result in recurrent corneal erosions, irregular corneal astigmatism, and decreased vision.

[6] Epithelial basement membrane dystrophy (EBMD) is caused by genetic mutations, also known as pathogenic variants. [7] Genetic mutations can be hereditary, when parents pass them down to their children, or they may occur randomly when cells are dividing.

Additional Characteristics

  • Epithelial basement membrane dystrophy
  • Map-dot-fingerprint dystrophy
  • Cogan microcystic epithelial dystrophy

Signs and Symptoms

Common Signs and Symptoms of Epithelial Basement Membrane Dystrophy

Epithelial basement membrane dystrophy (EBMD) can manifest in various ways, affecting individuals differently. Here are some common signs and symptoms associated with this condition:

  • Recurrent Corneal Erosions: One of the most characteristic symptoms of EBMD is recurrent corneal erosions, which occur when the epithelium breaks down and exposes the underlying basement membrane (1, 3, 6).
  • Blurred Vision: Many patients experience blurred vision due to irregular astigmatism or other visual disturbances caused by the condition (4, 7, 8).
  • Sensitivity to Light: Some individuals may feel sensitive to light, which can be a symptom of EBMD (5, 9).
  • Dry Eyes: Dry eyes are another common complaint among patients with EBMD (10).
  • Pain and Discomfort: In some cases, the condition can cause pain and discomfort in the eye, particularly when the corneal erosions occur (11).

It's essential to note that many people with EBMD may not experience any symptoms at all, or their symptoms may be mild. However, if you're experiencing any of these signs and symptoms, it's crucial to consult an eye care professional for proper evaluation and treatment.

References:

[1] Context 2 [3] Context 3 [6] Context 12 [4] Context 5 [7

Additional Symptoms

Diagnostic Tests

Epithelial basement membrane dystrophy (EBMD) can be diagnosed through various diagnostic tests and procedures.

Clinical Diagnosis The clinical diagnosis of EBMD is primarily based on the patient's history and physical examination [1]. A thorough eye exam, including a slit-lamp examination, is essential to identify characteristic findings such as diffuse gray opacities in varying patterns, irregular, thickened, multilayered, and folded epithelial basement membrane [7].

Diagnostic Procedures While no specific procedures are required for the diagnosis of EBMD, several tests can be used to support the clinical diagnosis:

  • Corneal Topography: This diagnostic test measures the smoothness and contour of the corneal surface, which is often abnormal in patients with EBMD [6].
  • Slit-Lamp Exam: A slit-lamp exam is a crucial part of the eye examination, allowing for a detailed inspection of the eyes using high-intensity light. It can reveal characteristic findings such as diffuse gray opacities and irregular epithelial basement membrane [8].

Other Diagnostic Tests While not typically used for diagnosing EBMD, other tests may be employed to rule out other conditions or assess the severity of the disease:

  • Corneal Mapping: This test is not generally used for diagnosing EBMD but can be useful in assessing the extent of corneal involvement [5].
  • Genetic Tests: Genetic testing may be available for certain cases, particularly when a family history of EBMD is present [2].

It's essential to consult with an eye care professional or a specialist in ophthalmology for accurate diagnosis and treatment planning.

References: [1] - Context 1 [2] - Context 2 [5] - Context 5 [6] - Context 6 [7] - Context 7 [8] - Context 8

Additional Diagnostic Tests

  • Corneal Topography
  • Genetic Tests
  • Slit-Lamp Exam
  • Corneal Mapping

Treatment

Treatment Options for Epithelial Basement Membrane Dystrophy

Epithelial basement membrane dystrophy (EBMD) is a corneal disorder that can cause recurrent epithelial erosions and visual disturbances. While there is no cure for EBMD, various treatment options are available to manage the symptoms and prevent recurrence.

Medical Therapy

According to [4], combination therapy involving doxycycline and methylprednisolone has been shown to inhibit matrix metalloproteinase-9, which is implicated in cleaving scaffolding proteins. This can help reduce the frequency of corneal erosions.

  • Topical cocaine-assisted epithelial debridement offers a simple and inexpensive method for treating patients with ABMD who experience recurrent corneal erosions [6].
  • Muro 128 (Bausch + Lomb) is a medication that comes in both drop and ointment forms, which can help draw water out of the cornea and reduce inflammation [1].

Other Treatment Options

In addition to medical therapy, other treatment options for EBMD include:

  • Manual superficial keratectomy: This surgical procedure involves removing the affected epithelial layer from the cornea. According to [8], this treatment can be effective in reducing the frequency of corneal erosions.
  • Phototherapeutic keratectomy (PTK): PTK is a laser-based treatment that can help remove damaged tissue and promote healing. According to [7], PTK has been shown to be effective in treating EBMD.

Recurrent Corneal Erosions

Unfortunately, there is no cure for EBMD, and corneal erosions can recur even after treatment. According to [5], repeat surgery may be necessary to treat recurrent corneal erosions.

References:

[1] Muro 128 (Bausch + Lomb) medication information [4] Combination therapy for epithelial basement membrane dystrophy [6] Topical cocaine-assisted epithelial debridement for ABMD [7] Phototherapeutic keratectomy for epithelial basement membrane dystrophy [8] Manual superficial keratectomy for EBMD

๐Ÿ’Š Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.

Differential Diagnosis

Epithelial Basement Membrane Dystrophy (EBMD) Differential Diagnosis

Epithelial basement membrane dystrophy, also known as map-dot-fingerprint corneal dystrophy, is a condition that affects the cornea and can be challenging to diagnose. To determine the correct diagnosis, it's essential to consider the differential diagnoses for EBMD.

Possible Differential Diagnoses:

  • Fungal keratitis: This is an infection of the cornea caused by fungi, which can present with similar symptoms to EBMD.
  • Bacterial keratitis: A bacterial infection of the cornea that can cause inflammation and damage to the corneal tissue.
  • Viral keratitis: An infection of the cornea caused by viruses, such as herpes simplex virus, which can lead to inflammation and scarring.
  • Parasitic keratitis: A rare condition where parasites infect the cornea, causing inflammation and damage.

Key Diagnostic Features:

To differentiate EBMD from these conditions, several key features should be considered:

  • Clinical presentation: Patients with EBMD may present with symptoms such as blurry or fluctuating vision, foreign body sensation, irritation, and redness.
  • Histopathological findings: Histopathology of the cornea in patients with EBMD shows thickening of the Descemet membrane and endothelial atrophy, amyloid deposits, and spheroidal degeneration.
  • Imaging studies: Imaging studies such as slit-lamp biomicroscopy and confocal microscopy can help to confirm the diagnosis.

References:

  • [3] Epithelial basement membrane dystrophy (EBMD) is an anterior corneal dystrophy that can be asymptomatic or cause blurred vision, recurrent corneal erosion or ...
  • [5] by C Constantin ยท 2021 ยท Cited by 5 โ€” Histopathologically: thickening of the Descemet membrane and endothelial atrophy, amyloid deposits, and spheroidal degeneration.
  • [8] The most common corneal dystrophy is epithelial basement membrane dystrophy. ... Establish differential diagnosis (fungal, bacterial, viral, parasitic).
  • [9] When the basement membrane develops abnormally, the epithelial cells cannot properly adhere to it. This, in turn, causes recurrent epithelial erosions, in ...

Note: The above information is based on the search results provided and may not be an exhaustive list of differential diagnoses or diagnostic features for EBMD.

Additional Information

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disease_ontology
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2015-10-16T17:17:01Z
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elvira
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ORDO:98956
IAO_0000115
An epithelial and subepithelial dystrophy that is characterized by sheet-like areas of basement membrane originating from the basal epithelial cells of the corneal epithelium and extending superficially into the epithelium.
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microcystic corneal dystrophy
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