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Purpose To describe 7 patients with paraproteinemic keratopathy and to highlight the clinical and pathologic diversity of this rare entity and the importance of timely, systemic evaluation. Design Retrospective, multicenter collaborative case series. Participants Seven patients with paraproteinemic keratopathy. Methods Clinical and pathologic records were reviewed to identify patients with well-documented corneal immunoglobulin deposits. Detailed ophthalmologic and medical histories were assembled. In 6 patients, corneal tissue was evaluated histochemically and immunohistochemically; in selected cases, corneal tissue was evaluated by in situ hybridization and ultrastructurally. Main Outcome Measures Visual acuity and anterior segment examination at presentation and follow-up; local therapy; systemic diagnosis and management; and histopathologic, immunohistochemical, in situ hybridization, and ultrastructural findings. Results Seven patients were identified with corneal immunoglobulin deposition. In addition to previously reported crystalline, nummular, patch-like, and lattice-like corneal opacities, prominent corneal vascularization was present in 2 patients mimicking interstitial keratitis and limbal stem cell deficiency. All patients had evidence of paraproteinemia in a setting of monoclonal gammopathy of undetermined significance, smoldering plasma cell myeloma, or Waldenstrom macroglobulinemia. Corneal findings were the first manifestation of systemic disease in 4 patients, and the diagnosis was not suspected in 3 of these patients. Pathologic evaluation of biopsied corneal and conjunctival tissues demonstrated immunoglobulin deposits. Previously unreported ultrastructural patterns in the cornea were noted: large scroll-like immunotactoid deposits, immune complex-like deposits, and randomly arranged fibrils morphologically intermediate between amyloid and immunotactoid deposits. Surgical intervention to improve vision was performed in 4 patients, with recurrence of deposits in 3 patients. Three patients underwent systemic therapy with diminution of the deposits and improvement in vision in 1 patient. Conclusions The clinical and pathologic expressions of corneal immunoglobulin deposits are protean and present a diagnostic challenge. Early recognition of this rare entity is important to address the potentially serious associated systemic disease.

Purpose To evaluate the efficacy and safety of daclizumab therapy for patients with various ophthalmologic inflammatory disorders (all having previously failed standard treatment methods). Design Retrospective, nonrandomized case series. Participants Fourteen patients. Methods Fourteen patients were treated with daclizumab after previously failing standard treatment methods. Main outcome measures Inflammation and visual acuity. Results Twelve of 27 (44%) eyes and 5 of 14 (36%) patients had improvement in visual acuity; 9 of 27 (33%) eyes and 5 of 14 (36%) patients had no change in visual acuity; and 6 of 27 (22%) eyes and 4 of 14 (27%) patients had continued visual loss. Based on degree of inflammation, 16 of 27 eyes (59%) had improvement, 3 of 27 (11%) eyes had no change, and 8 of 27 (30%) eyes worsened. Conclusions Daclizumab is safe and, at least in some patients, appears to be an effective medication in the treatment of ocular inflammatory disorders.

Purpose To describe an unusual and possibly new keratopathy in human immunodeficiency virus (HIV)-positive patients. Design Retrospective observational case series. Participants: Six HIV-positive patients at four centers. Methods A complete medical history was obtained and a thorough ophthalmic examination was performed on each of the patients. Results Each patient had bilaterally symmetrical keratopathy consisting of variably pigmented round and reticular posterior intracorneal precipitates at the level of Descemet’s membrane. The precipitates were diffuse, but larger and more prominent near the limbus and finer toward the central cornea. None of the eyes were inflamed, and all had normal vision. No patient had uveitis on presentation or any history of cytomegalovirus retinitis. Conclusion These patients have a unique posterior keratopathy, which requires further investigation.