SCLERITIS AND MUCOSAL-ASSOCIATED LYMPHOID-TISSUE LYMPHOMA - A NEW MASQUERADE SYNDROME

Purpose: To present a new masquerade syndrome showing features of muco sal-associated lymphoid tissue (MALT) lymphoma associated with choroid al white dots and scleritis. Differentials including systemic lymphoma , central nervous system lymphoma, and etiologies of white-dot syndrom es and scleritis are discussed. Patients and Methods: A 42-year-old ma n who had decreased vision and ocular redness of his right eye for 4 y ears had a biopsy-proven diffuse anterior and posterior scleritis asso ciated with intense circumferential perilimbal chemosis and ipsilatera l yellow-white choroidal dots. A new conjunctival biopsy was performed because of unresponsiveness to high-dose systemic steroid and cycloph osphamide therapy. Immunostains for lymphocyte markers were performed. Results: A morphologically and immunohistochemically typical, monotyp ical mukappa immunoglobulin light chain secreting B-cell MALT-lymphoma was diagnosed. Eighteen months after completion of radiotherapy, the patient recovered completely, except for the choroidal dots, which rem ained unchanged. Conclusion: When scleritis, even histologically prove n, fails to respond to immunosuppressive therapy, a new biopsy is mand atory to rule out a misdiagnosed MALT lymphoma.