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Published on: March 7, 2019
Secondary amyloidosis in the hydrops lesion of a patient with pellucid marginal degeneration
Hugo Y Hsu1, Nancy J Phillips, George J Harocopos
1Department of Ophthalmology, St. Louis University School of Medicine, St. Louis, MO, USA.
Purpose:
To report a case of secondary corneal amyloidosis in a chronic hydrops lesion of a patient with pellucid marginal degeneration (PMD).
Methods:
Clinicopathologic case report with a review of the literature. A 63-year-old man with PMD developed acute hydrops in 1999, which never resolved and became an elevated, gelatinous lesion with peripheral neovascularization. Penetrating keratoplasty was performed in 2005, and the excised button was examined histopathologically.
Results:
The excised button revealed substantial subepithelial acellular deposits consistent with amyloid in the area of the unresolved gelatinous lesion, overlying a disruption in Descemet membrane, confirming the diagnosis of secondary corneal amyloidosis.
Conclusions:
Secondary corneal amyloidosis is a corneal response to chronic injury and irritation that is not commonly reported and often overlooked. To the best of our knowledge, this is the first report of a dramatic secondary amyloidosis deposit in the chronic hydrops lesion of a patient with PMD.
Insights
This study reports a rare case of secondary corneal amyloidosis in a patient with pellucid marginal degeneration (PMD). The condition developed in a chronic hydrops lesion, highlighting a unique corneal response to injury.
Area of Science:
- Ophthalmology
- Pathology
Background:
- Pellucid marginal degeneration (PMD) is a rare, progressive corneal thinning disorder.
- Corneal hydrops, characterized by stromal edema, can occur in PMD.
- Secondary corneal amyloidosis is an uncommon deposition of amyloid in the cornea.
Observation:
- A 63-year-old male with PMD developed persistent acute hydrops.
- The hydrops evolved into an elevated, gelatinous lesion with neovascularization.
- Histopathological examination of the excised cornea revealed amyloid deposits.
Findings:
- The excised corneal button showed significant subepithelial amyloid deposits.
- Amyloid deposition was associated with a disrupted Descemet membrane.
- This confirmed the diagnosis of secondary corneal amyloidosis in the context of chronic hydrops in PMD.
Implications:
- This case represents the first documented instance of significant secondary amyloidosis in a chronic hydrops lesion associated with PMD.
- Secondary corneal amyloidosis may be an underdiagnosed complication of chronic corneal injury.
- Further research is warranted to understand the pathogenesis and clinical significance of this condition.
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