Hereditary Benign Intraepithelial Dyskeratosis

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Abstract
The disease described here is considered to be a previously unreported entity affecting mucous membranes of the eye and mouth. The eye lesions occur bilaterally and involve the interpalpebral conjunctiva; in the mouth there are alterations of the buccal mucosa. Typical changes at these sites may be observed in early childhood and persist throughout life with variable intensity. The clinical picture shows considerable diversity, but the histopathology has characteristics common to both lesions and thus supports the clinical impression of a single disease process. There is no sex predominance and as far as is known the disease is inherited as a simple Mendelian dominant. The present report is concerned primarily with the ophthalmologic aspects of this disease. Information on the oral manifestations of this dyskeratosis and summary data on its hereditary transmission are given in a second paper in this series by C. J. Witkop Jr., C. H. Shankle, J.

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