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An immunohistochemical analysis and comparison of posterior polymorphous dystrophy with congenital hereditary
Glenn C Cockerham1, Nora V Laver, Ahmed A Hidayat
1Department of Ophthalmic Pathology, Armed Forces Institute of Pathology, Washington, DC, USA. gcockerham@prodigy.net
Purpose:
To evaluate the immunohistochemical profiles of the abnormal endothelial cells of posterior polymorphous dystrophy (PPMD) and congenital hereditary endothelial dystrophy (CHED).
Methods:
Formalin-fixed, paraffin-embedded sections of seven corneas with the diagnosis of PPMD (seven patients), six corneas with the diagnosis of CHED (four patients), and five control corneas were stained with hematoxylin-eosin. Adjacent histologic sections were stained with monoclonal antibodies that react with pancytokeratin, AE1/AE3, cytokeratin (CK) 7, CK 20, CAM 5.2, and epithelial membrane antigen. The immunoreactivity of the corneal endothelium was assessed by light microscopy.
Results:
The endothelial cells stained positive for pancytokeratin and CK 7 in seven of seven corneas of patients with PPMD and five of six corneas of patients with CHED; variable positivity was seen to AE1, AE3, and CAM 5.2. The endothelium was uniformly negative to staining by CK 20. The epithelium stained positive with pancytokeratin, AE1, and AE3. All control corneas were negative for pancytokeratin, CK 7, and CK 20.
Conclusion:
The abnormal endothelium in both PPMD and CHED expresses similar CKs, including CK 7, which is not present in normal endothelium or surface epithelium. This may indicate a shared developmental abnormality in these conditions, as previously suggested by ultrastructural studies and genetic mapping.
Insights
Abnormal endothelial cells in posterior polymorphous dystrophy (PPMD) and congenital hereditary endothelial dystrophy (CHED) share similar keratin profiles, including CK 7, suggesting a common developmental origin for these corneal conditions.
Area of Science:
- Ophthalmology
- Cell Biology
- Histopathology
Background:
- Posterior polymorphous dystrophy (PPMD) and congenital hereditary endothelial dystrophy (CHED) are inherited corneal disorders.
- The specific cellular and molecular mechanisms underlying these conditions are not fully understood.
- Investigating the immunohistochemical characteristics of abnormal endothelial cells can provide insights into their pathogenesis.
Purpose of the Study:
- To compare the immunohistochemical profiles of abnormal corneal endothelial cells in PPMD and CHED.
- To identify specific markers that differentiate or link these two endothelial dystrophies.
- To explore potential shared developmental pathways.
Main Methods:
- Corneal tissue samples from patients with PPMD, CHED, and control subjects were analyzed.
- Immunohistochemical staining was performed using a panel of monoclonal antibodies against various keratins (pancytokeratin, AE1/AE3, CK 7, CK 20) and epithelial membrane antigen.
- Immunoreactivity of corneal endothelial cells was assessed via light microscopy.
Main Results:
- Endothelial cells in both PPMD and CHED samples showed positive staining for pancytokeratin and cytokeratin 7 (CK 7).
- Variable positivity was observed for AE1, AE3, and CAM 5.2, while CK 20 staining was uniformly negative.
- Normal corneal endothelium and epithelium did not stain positive for CK 7, and control corneas were negative for pancytokeratin, CK 7, and CK 20.
Conclusions:
- The abnormal endothelium in PPMD and CHED exhibits similar keratin expression patterns, notably including CK 7.
- The presence of CK 7 in abnormal endothelium, but not in normal endothelium or epithelium, suggests a shared characteristic.
- These findings support the hypothesis of a common developmental abnormality in PPMD and CHED, consistent with prior ultrastructural and genetic studies.
