Four members of a family were affected with lattice dystrophy of the cornea. One of them showed recurrence of the disease in the corneal graft in one eye 9 years after a keratoplasty, and a very advanced stage of the disease in the fellow eye. Therefore a keratoplasty was done in this eye. The corneal button was thoroughly investigated by means of histochemistry, electron microscopy and X-ray electron microanalysis. Two young members of the family showing early signs of the disease were followed up for 2 years. It has been possible to observe the evolution of their lesions. Histopathological examinations revealed the presence of two different types of abnormal deposits under the epithelium and in the stroma. X-ray electron microanalysis of the stromal deposits revealed a substantial increase in sulphur content.

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