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January 2002

Epithelial Origin of "Stromal" Corneal Dystrophies

Arch Ophthalmol. 2002;120(1):101. doi:

We read with great interest the editorial by Dunaief et al in the January 2001 issue of the ARCHIVES, which discussed the epithelial genesis of most of the classical "stromal dystrophies" of the cornea, their ensuing reclassification, and new therapeutic approaches.1 We were, however, astonished to find that our early paper dealing with the same question2 is cited by the authors as supporting a fibroblastic origin of the stromal deposits. As a matter of fact, we were among the first, if not the first, who, after light-microscopic and electron-microscopic studies of early recurrent granular dystrophy, postulated an epithelial origin of the deposits in granular and also in lattice dystrophy. The last sentence of our summary reads, "The clinical pictures and microscopic findings strongly suggest that granular dystrophy may primarily be an epithelial not a stromal disease." This suggestion was at that time strongly opposed by most experts but was adopted by Johnson et al in their 1981 paper.3 We were able to further support our hypothesis with additional morphological and immunohistochemical findings.4,5 But because the responsible gene and its product were not yet identified, an involvement of the keratocytes, at least in the late stages of the disease, could not be completely excluded. We found, however, the results indicating an epithelial origin convincing enough to start a surgical pilot study as early as 1995. We used the then recently developed variant of limbokeratoplasty to prevent dystrophy recurrences by transplanting donor stem cells together with a clear donor cornea.6 The initial results of this study, which will have to go on for many more years to yield valid results, are correctly but incompletely cited in the editorial. The credit for the final proof of the epithelial genesis certainly goes to those who revealed the molecular genetic basis and thus definitely opened the way to new therapeutic options. Nevertheless, the older work should also be cited correctly.

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