A comeal specimen obtained by surgery in a 55-year-old woman with Chandler’s syndrome was studied by light and transmission electron microscopy as well as by immunocytochemistry. The pathologic features were abnormalities of the endothelially derived cells lining the posterior comeal surface with a fibrous material consisting of collagen fibrils, filaments and banded material similar to that found in the anterior part of Descemet’s membrane observed between the endothelial cell layer and Descemet’s membrane. The multilayered endothelial cell layer in our case was found to be strongly positive for cytokeratins K7 and KL1 and vimentin and negative for factor-VIII-related antigen, neuron-specific enolase, nerve tissue S–100 protein, epithelial membrane antigen, CD 68, actin and desmin. This immunohistochemical reaction pattern argues in favor of an epithelial origin for cells lining the endothelial cell layer in Chandler’s syndrome.

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