A one-year-old girl with Letterer-Siwe disease developed bilateral perforating corneal ulcers. She underwent penetrating keratoplasty 16 years later, utilizing a donor cornea taken from a patient who died with Letterer-Siwe disease. Examination of the posterior segments of the donor's eyes revealed bilateral choroidal infiltration by the histiocytic disease process. Similar intraocular involvement in Letterer-Siwe disease, predominantly in the posterior uveal tract, was reported previously in eight patients. Uveal involvement is also seen in two similar clinicopathologic entities-juvenile xanthogranuloma and familial erythrophagocytic lymphohistiocytosis. Thus, thorough ophthalmic evaluation of all patients afflicted with Letterer-Siwe disease is of utmost importance.
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