Ophthalmic findings in scleromyxedema.

PURPOSE: To catalog ophthalmic findings in a cohort of patients with scleromyxedema.

METHODS

Thirty-five biopsy-proven patients with scleromyxedema evaluated at the Mayo Clinic in Rochester, Minnesota, from 1960 to 1991 were identified. Eye examinations were performed on 17 of the patients. Ophthalmic findings not attributable to other systemic or ocular disease were recorded.

RESULTS

The following abnormalities were considered secondary to scleromyxedema: corneal opacities (2 patients), thickened eyebrow or eyelid skin (4 patients), lagophthalmos (1 patient), and ectropion (2 patients). One patient who had concurrent polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome also had choroidal folds and papilledema. A corneal biopsy in one patient disclosed deposits of acid mucopolysaccharide, consistent with one of two previously published cases.

CONCLUSION

A series of patients with scleromyxedema was reviewed. This systemic disorder infrequently may cause visually significant ophthalmic manifestations, including eyelid changes and corneal deposits. Further study is needed to characterize better the nature of the corneal opacities.