The keratoconus enigma: A review with emphasis on pathogenesis

Purpose: To review the literature on the etiopathogenesis of keratoconus (KC). Methods: A literature search was conducted using PUBMED and Google Scholar for keratoconus. The authors analyzed epidemiology studies, reviews, and case reports. Results: Atopy and ocular surface inflammation are a common features of KC and should lead to a reconsideration of the traditional definition of KC as a “non inflammatory” condition. Co-morbidities suggest that KC may be the ocular manifestation of a systemic disease. Finally, KC shows higher prevalence in certain ethnicities, which calls into question the status of KC as a rare disease, at least in these communities. Conclusion: We believe that future studies should test whether selected, high prevalence populations exhibit specific genetic background and/or ethno-specific environmental risk factors.