Boehm Syndrome
Encyclopedia
Boehm Syndrome is a symptom-specific map-dot-fingerprint dystrophy
of the cornea
.
The epithelium
is separated from the stroma by a membrane
. In map-dot-fingerprint dystrophy, this membrane develops abnormally exhibiting a map-like appearance or perhaps opaque dots. Sometimes lines are formed that resemble small fingerprints. In some cases, this irregular structure can lead to painful epithelial erosions.
endings, causing moderate to severe pain for hours or days. Once the erosions begin, even periods of brief sleep can exacerbate the problem.
Patients may describe the sensation as "my eyelids stick to my eyes when I try to open them, which is immediately followed by intense pain". In some cases, this syndrome may disappear after one or more episodes. For others, it may be a lifelong disease.
There has been some success with the use of highly permeable extended-wear contact lenses worn only during sleep. These lenses, often referred to as "bandage lenses", or "contact bandages" are prescribed with the lowest available prescription, as they simply act as a buffer between the surface of the cornea
and the eyelids.
Dystrophy
Dystrophy is any condition of abnormal development, often denoting the degeneration of muscles.-Types:* Muscular dystrophy* Duchenne muscular dystrophy* Becker's muscular dystrophy* Reflex neurovascular dystrophy* Retinal dystrophy* Conal dystrophy...
of the cornea
Cornea
The cornea is the transparent front part of the eye that covers the iris, pupil, and anterior chamber. Together with the lens, the cornea refracts light, with the cornea accounting for approximately two-thirds of the eye's total optical power. In humans, the refractive power of the cornea is...
.
The epithelium
Epithelium
Epithelium is one of the four basic types of animal tissue, along with connective tissue, muscle tissue and nervous tissue. Epithelial tissues line the cavities and surfaces of structures throughout the body, and also form many glands. Functions of epithelial cells include secretion, selective...
is separated from the stroma by a membrane
Biological membrane
A biological membrane or biomembrane is an enclosing or separatingmembrane that acts as a selective barrier, within or around a cell. It consists of a lipid bilayer with embedded proteins that may constitute close to 50% of membrane content...
. In map-dot-fingerprint dystrophy, this membrane develops abnormally exhibiting a map-like appearance or perhaps opaque dots. Sometimes lines are formed that resemble small fingerprints. In some cases, this irregular structure can lead to painful epithelial erosions.
Symptoms
In Boehm Syndrome, recurrent epithelial erosions occur only when the eyelids are opened after some period of sleep. Recurrent corneal erosions may happen at any time of night or day. Boehm Syndrome does not. It occurs only when the inside of the eyelid tears away epithelial cells after minutes or hours of sleep. This may expose nerveNerve
A peripheral nerve, or simply nerve, is an enclosed, cable-like bundle of peripheral axons . A nerve provides a common pathway for the electrochemical nerve impulses that are transmitted along each of the axons. Nerves are found only in the peripheral nervous system...
endings, causing moderate to severe pain for hours or days. Once the erosions begin, even periods of brief sleep can exacerbate the problem.
Patients may describe the sensation as "my eyelids stick to my eyes when I try to open them, which is immediately followed by intense pain". In some cases, this syndrome may disappear after one or more episodes. For others, it may be a lifelong disease.
Treatment
Treatment may include some of the standard procedures used on corneal erosions, like corneal abrasion.There has been some success with the use of highly permeable extended-wear contact lenses worn only during sleep. These lenses, often referred to as "bandage lenses", or "contact bandages" are prescribed with the lowest available prescription, as they simply act as a buffer between the surface of the cornea
Cornea
The cornea is the transparent front part of the eye that covers the iris, pupil, and anterior chamber. Together with the lens, the cornea refracts light, with the cornea accounting for approximately two-thirds of the eye's total optical power. In humans, the refractive power of the cornea is...
and the eyelids.
External links
- Facts About the Cornea and Corneal Disease The National Eye Institute (NEI)
- Boehm Syndrome Webster's Online Dictionary
- Recurrent Corneal Erosion St. Luke's