Central cloudy dystrophy of François: a case series and literature review of a rare corneal disease

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Daniel Bastan-Fabian, MD
Brenda Ventura-Gomez, MD
Julio C. Jimenez-Perez, MD
Enrique Sanchez-Maldonado, MD
Alejandro Rodriguez-Garcia, MD

Abstract

Central cloudy dystrophy of François (CCDF) is a rare corneal dystrophy characterized by bilateral, symmetric, gray, cloudlike lesions in the central posterior stroma. Diagnosis relies on detailed family history and careful slit-lamp examination to differentiate it from Vogt posterior crocodile shagreen. We describe 5 female siblings from two families (mean age, 58.5 years) with typical bilateral lesions consistent with CCDF. Very few cases of CCDF have been reported, and its pathophysiology and relationship to other corneal stromal dystrophies or degenerations remain poorly understood. These 5 cases constitute the second-largest series and the first documented reports of CCDF in the Mexican population, supported by representative illustrations and accompanied by a comprehensive literature review.

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How to Cite
1.
Bastan-Fabian D, Ventura-Gomez B, Jimenez-Perez JC, Sanchez-Maldonado E, Rodriguez-Garcia A. Central cloudy dystrophy of François: a case series and literature review of a rare corneal disease. Digit J Ophthalmol. Published online September 24, 2026. doi:10.5693/djo.02.2026.05.004
Section
Case Reports
Author Biography

Alejandro Rodriguez-Garcia, MD, Tecnologico de Monterrey, School of Medicine and Health Sciences, Institute of Ophthalmology and Visual Sciences, Monterrey, Mexico

Dr. Alejandro Rodriguez-Garcia is a board-certified ophthalmologist by the Mexican Council of Ophthalmology. He received an honorific mention after graduating from medical school at Tecnologico de Monterrey, Mexico. He completed a postdoctoral research fellowship at the Hilles Immunology Laboratory of the Massachusetts Eye & Ear Infirmary, Harvard Medical School, where he worked in basic science and translational research on herpetic eye disease and other ocular surface and intraocular inflammatory disorders. Dr. Rodriguez-Garcia completed a Residency in ophthalmology at Tecnologico de Monterrey, where he became Chief Resident and graduated with highest honors. He then returned for a Clinical & Research Fellowship in Ocular Immunology & Uveitis at the Massachusetts Eye & Ear Infirmary, Harvard Medical School, under Dr. C. Stephen Foster´s mentorship, where he served as Chief Fellow. 

Dr. Rodriguez-Garcia is the former President of the College of Ophthalmologists in his home state of Coahuila, Mexico, and the former President of the Mexican Center of Ocular Inflammatory Disorders, which is affiliated with the Mexican Society of Ophthalmology.

He was appointed Clinical Professor of Ophthalmology in the residency program at Tecnologico de Monterrey in 1994, where he served as Senior Clinical Research Coordinator until 2021. From 2004 until 2021, he served as Academic Coordinator of the ophthalmology residency program at the same institution. From 2001 until now, he has been the Academic Director of the ophthalmology residency program at Tecnologico de Monterrey, Mexico.  

Dr. Rodriguez-Garcia is a Mexican System of Researchers (SNI) level-III member from the National Council of Research and Technology (CONAHCYT), an agency affiliated with the Mexico Federal Government. His research interests focused on diagnosing and treating different infectious and inflammatory eye diseases. He has made numerous scientific and educational contributions to ocular immunology, uveitis, and corneal and ocular surface disease through many courses, symposia, lectures, and publications in different peer-reviewed journals worldwide.

References

Soh YQ, Kocaba V, Weiss JS, et al. Corneal dystrophies. Nat Rev Dis Primers 2020;6:46.

Weiss JS, Møller HU, Aldave AJ, et al. IC3D classification of corneal dystrophies-edition 2. Cornea 2015;34:117-59.

François J. New hereditary dystrophy of the cornea [in Frence]. Bull Soc Belge Ophtalmol 1956;111:391-9.

François J, Neetena A. New hereditary-familial dystrophy of the corneal parenchyma (spotted hereditary dystrophy) [in French]. Bull Soc Belge Ophtalmol 1957;114:641-6.

Klintworth GK. Corneal dystrophies. Orphanet J Rare Dis 2009;4:7.

Kobayashi A, Sugiyama K, Huang AJ. In vivo confocal microscopy in patients with central cloudy dystrophy of François. Arch Ophthalmol 2004;122:1676-9.

Utine CA, Oral D, Altunsoy M, et al. In vivo confocal microscopy in presumed central cloudy dystrophy of François. Ophthalmic Surg Lasers Imaging Retina 2010;3:1-4.

Meyer JC, Quantock AJ, Thonar EJ, et al. Characterization of a central corneal cloudiness sharing features of posterior crocodile shagreen and central cloud dystrophy of François. Cornea 1996;15:347-54.

Hidalgo‐Bravo A, Acosta‐Nieto ML, Normendez‐Martinez MI, et al. Dermochondrocorneal dystrophy (François syndrome) in a Mexican patient and literature review. Am J Med Genet A 2016;170:446-51.

Bierly JR, George SP, Volpicelli M. Dermochondral corneal dystrophy (of François). Br J Ophthalmol 1992;76:760-1.

Caputo R, Sambvani N, Monti M, et al. Dermochondrocorneal dystrophy (François’ syndrome). Report of a case. Arch Dermatol 1988;124:424-8.

Weiss JS, Rapuano CJ, Seitz B, et al. IC3D Classification of Corneal Dystrophies—Edition 3. Cornea 2024;43:466-527.

Belliveau MJ, Brownstein S, Agapitos P, et al. Ultrastructural features of posterior crocodile shagreen of the cornea. Surv Ophthalmol 2009;54:569-75.

Strachan IM. Cloudy central corneal dystrophy of Francois: five cases in the same family. Br J Ophthalmol 1969;53:192-4.

Bramsen T, Ehlers N, Baggesen LH. Central cloudy corneal dystrophy of François. Acta Ophthalmol 1976;54:221-6.

Karp CL, Scott IU, Green WR, et al. Central cloudy corneal dystrophy of Francois: a clinicopathologic study. Arch Ophthalmol 1997;115:1058-62.

Zaidi A, McLeod SD. Laser in situ keratomileusis in a patient with presumed central cloudy corneal dystrophy of Francois. Am J Ophthalmol 2005;139:376-7.

Woodward M, Randleman JB, Larson PM. In vivo confocal microscopy of polymorphic amyloid degeneration and posterior crocodile shagreen. Cornea 2007;26:98-101.

Seitz B, Lisch W, Weiss J. The revised newest IC³D classification of corneal dystrophies [in German]. Klin Monbl Augenheilkd 2015;232:283-94.

Fernandez-Sasso D, Acosta JE, Malbran E. Punctiform and polychromatic pre-Descemet’s dominant corneal dystrophy. Br J Ophthalmol 1979;63:336-8.

Macsai MS, Doshi H. Clinical pathologic correlation of superficial corneal opacities in X-linked ichthyosis. Am J Ophthalmol 1994;118:477-84.

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