Epidermolysis bullosa

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2. Ophthalmology

Ophthalmology is the medical-surgical specialty that studies the structure and function of the eye. Like the skin, the eye's surface is in contact with the external environment, necessitating a series of protective mechanisms. These mechanisms include the eyelids and cornea, among others, with these two being particularly problematic for individuals with EB. 

Eyelids 

The eyelids are movable folds of muscle and mucous skin that cover the upper and lower parts of the eye. Like any other skin surface on the body, the skin of the eyelids can also be affected, leading to blisters and erosions. 

In more advanced cases, ectropion may develop, a condition in which the eyelid turns outward away from the eye, leaving the inner surface exposed and preventing the eyelid from fully closing. Ectropion occurs due to repetitive scarring that causes retraction of the eyelid skin. This condition can be very uncomfortable for the patient; when the eyelids remain partially open (a situation known as lagophthalmos), it can lead to increased dryness and other ocular problems. Patients often experience a constant sensation of a foreign body and dry eyes, which can typically be addressed through surgical intervention. 

Additionally, chronic inflammation of the eyelid margins, known as blepharitis, can occur. This inflammation results from the blockage of small glands located at the base of the eyelashes, which are responsible for lubrication. The consequences include irritation, redness, and a drier eye that is more susceptible to erosions. 

Cornea and conjunctiva 

The other structures that may also be affected are those that form the eye's surface: the cornea and the conjunctiva. The cornea is the transparent, anterior part of the eye, and it connects peripherally to the conjunctiva, a transparent layer that covers the outer surface of the eyeball and the inner part of the upper and lower eyelids. Both structures have an internal structure similar to that of the skin, making them susceptible to blister and erosion formation from minimal contact. These blisters often rupture due to the constant friction from the eyelids during blinking, leading to corneal erosion, which is essentially the loss of the corneal epithelium in the area where the blister formed. 
The cornea is likely the most sensitive structure in the body, containing a high concentration of nerve endings, making injuries to it extremely painful. This corneal hypersensitivity serves as a defense mechanism, allowing for the quick detection of minor external irritations so that the eyelids can close to prevent further damage. In addition to pain, symptoms of corneal injuries may include photophobia (light sensitivity), tearing, eyelid edema (swelling), and redness of the eye. 

 

conjuntivitis

 

Corneal blister formation occurs more frequently in children and decreases in incidence as individuals with EB grow older. It is also important to note that fewer tears are produced at night, leading to a drier ocular surface. This dryness can cause the eyelids to stick to the cornea, and when the eyes are opened in the morning, some of the corneal epithelium may be dragged along, resulting in erosion. For this reason, the use of lubricants and eye drops is recommended as a preventive measure for individuals with EB. 
Once erosion occurs, it typically heals within 2 to 3 days without leaving scars. Usually, the ophthalmologist will prescribe an antibiotic ointment to prevent possible infections until the erosion has fully healed. Additionally, occluding the eyelid with a patch can help minimize discomfort from blinking, along with oral analgesics for pain relief. Wearing sunglasses when the patch is removed can also be beneficial. 
Most ophthalmic injuries are the direct or indirect consequences of blisters and erosions of the cornea and eyelids. Over time, and in more advanced cases, these blisters and erosions can lead to chronic complications in the eye: 
  • Corneal Scars: When a corneal erosion becomes infected, it can result in scarring of the corneal tissue, which appears as a white spot on the cornea (corneal opacification). If the scar is located at the periphery of the cornea, it generally does not affect vision. However, if it occurs in the central area (the pupil region), it can decrease visual acuity. 
  • Corneal Pannus Formation: This condition involves the growth of blood vessels over the cornea, accompanied by the presence of inflammatory tissue. Without proper treatment, it can also impair vision. 
  • Symblepharon: This refers to the fusion of the inner surfaces of the eyelids with the conjunctiva covering the eyeball, or even with the cornea. It occurs due to the frequent formation of injuries in the conjunctiva. If the mobility of the eyeball is compromised or vision is diminished due to corneal involvement, surgical intervention may be necessary to release the adhesions. 
  • Obstruction of the Tear Ducts: Injuries and scarring of the conjunctiva and eyelids can lead to obstruction of the eye's drainage ducts, resulting in constant tearing due to the inability to eliminate excess tears produced for lubrication. 
  • Astigmatism: Scarring on the eye can cause deformities in the surface of the eyeball, resulting in abnormal curvature of the cornea and distortion of images. Prescription glasses may be necessary to correct this vision problem. 
Last modified
04 April 2025