Purpose: We report a patient with progressive idiopathic, nonulcerative, noninflammatory, avascular, bilateral, paracentral and peripheral corneal thinning monitored for 13 years.

Methods: Because of progressive corneal thinning, the patient underwent several surgical procedures, including an arcuate lamellar keratectomy with suturing, bilateral 15-mm diameter onlay lamellar corneoscleral epikeratoplasties, and removal of interface epithelial tissue. Over time, the keratolysis also thinned the donor stroma, requiring a lamellar tectonic graft. A biopsy was performed of the patient's cornea and conjunctiva, and the tissue was analyzed for proteolytic enzymes.

Results: Increased quantities of matrix metalloproteinases (57 and 63 kDa) were extracted from the patient's normal-appearing and abnormal corneal samples but not from adjacent conjuctiva and sclera or normal controls. This is the first reported case with these clinical and laboratory findings.

Conclusion: A previously undescribed progressive idiopathic paracentral keratolysis is associated with increased quantities of matrix metalloproteinases. Clinical management requires tectonic corneal surgery.

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