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Lattice corneal dystrophy type I
Reviewed by Medicinsk Expert•Last updated: 2026-10-22•Condition

Lattice corneal dystrophy type I

Lattice corneal dystrophy type I is an inherited eye disorder characterized by the accumulation of amyloid deposits in the cornea, leading to vision impairment and painful corneal erosions.

Overview

Lattice corneal dystrophy type I is an eye disorder that affects the clear, outer covering of the eye called the cornea. The cornea must remain clear for an individual to see properly; however, in lattice corneal dystrophy type I, protein clumps known as amyloid deposits cloud the cornea, which leads to vision impairment. The cornea is made up of several layers of tissue, and in lattice corneal dystrophy type I, the deposits form in the stromal layer. The amyloid deposits form as delicate, branching fibers that create a lattice pattern.

Affected individuals often have recurrent corneal erosions, which are caused by separation of particular layers of the cornea from one another. Corneal erosions are very painful and can cause sensitivity to bright light (photophobia). Lattice corneal dystrophy type I is usually bilateral, which means it affects both eyes. The condition becomes apparent in childhood or adolescence and leads to vision problems by early adulthood.

Frequency

Lattice corneal dystrophy type I is one of the most common disorders in a group of conditions that are characterized by protein deposits in the cornea (corneal dystrophies); however, it is still a rare condition. The prevalence of lattice corneal dystrophy type I is unknown.

Causes

Lattice corneal dystrophy type I is caused by mutations in the TGFBI gene. This gene provides instructions for making a protein that is found in many tissues throughout the body, including the cornea. The TGFBI protein is part of the extracellular matrix, an intricate network that forms in the spaces between cells and provides structural support to tissues. The protein is thought to play a role in the attachment of cells to one another (cell adhesion) and cell movement (migration).

The TGFBI gene mutations involved in lattice corneal dystrophy type I change single protein building blocks () in the TGFBI protein. Mutated TGFBI proteins abnormally clump together and form amyloid deposits. However, it is unclear how the changes caused by the gene mutations induce the protein to form deposits.

Learn more about the gene associated with Lattice corneal dystrophy type I

  • TGFBI

Inheritance

This condition is inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder. In most cases, an affected person has one parent with the condition.

Other Names for This Condition

  • Biber-Haab-Dimmer dystrophy

Frequently Asked Questions

Early signs often appear in childhood or adolescence and can include blurry vision, sensitivity to bright light (photophobia), and episodes of severe eye pain, which indicate corneal erosions. If your child experiences these symptoms, especially recurrent eye pain or significant vision changes, it's important to consult an ophthalmologist for evaluation.
Over time, the amyloid deposits can significantly cloud the cornea, leading to progressive vision impairment and even blindness if untreated. The primary treatment to restore vision is a corneal transplant (keratoplasty), where the diseased cornea is replaced with a healthy donor cornea. While effective, there is a risk of recurrence of amyloid deposits in the transplanted cornea over many years.
Lattice corneal dystrophy type I is caused by mutations in the TGFBI gene and is inherited in an autosomal dominant pattern. This means that if one parent has the condition, each child has a 50% chance of inheriting the mutated gene and developing the disorder. Genetic testing for the TGFBI gene is available and can confirm the diagnosis and identify carriers within a family, which can be helpful for family planning.
Painful corneal erosions are often treated with lubricating eye drops or ointments, bandage contact lenses, and sometimes topical antibiotics to prevent infection. In severe cases, a procedure called anterior stromal puncture or phototherapeutic keratectomy (PTK) may be considered. While complete prevention is difficult due to the underlying condition, consistent use of lubricating eye drops can help maintain corneal surface integrity and potentially reduce the frequency of erosions.
Source: MedlinePlus, National Library of Medicine

MedlinePlus brings together authoritative health information from the National Library of Medicine (NLM), the National Institutes of Health (NIH), and other government agencies and health-related organizations.

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