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Morton F. Goldberg, MD

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The February 2008 article¹ on sialidosis (mucolipidosis type 1) correctly states that a characteristic duo of clinical findings (cherry-red spot in the macula as well as haze in the corneal stroma) occurs in this storage disease, combining the individual clinical signs that characterize other storage disorders; specifically, the sphingolipid storage diseases (typically, cherry-red spot) and the mucopolysaccharide storage diseases (typically, corneal haze).

Corneal haziness was not described in the February publication, but, as the original article describing sialidosis showed,² stromal haze is integral to the syndrome, just as macular cherry-red spot is. The mildness of the corneal haze results in its easily being overlooked in sialidosis. It would be of interest to know, therefore, if the case of sialidosis reported in the first reference did or did not have subtle haziness of the corneal stroma in addition to the obvious cherry-red spot in the macula.

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RELATED LETTER

Macular Cherry-Red Spot and Corneal Haze in Sialidosis (Mucolipidosis Type 1)—Reply
Charles C. Barr, J. Wesley Heroman, and Paul Rychwalski
Arch Ophthalmol. 2008;126(12):1778.
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