Scleritis and mucosal-associated lymphoid tissue lymphoma: a new masquerade syndrome

To present a new masquerade syndrome showing features of mucosal-associated lymphoid tissue (MALT) lymphoma associated with choroidal white dots and scleritis. Differentials including systemic lymphoma, central nervous system lymphoma, and etiologies of white-dot syndromes and scleritis are discussed.

A 42-year-old man who had decreased vision and ocular redness of his right eye for 4 years had a biopsy-proven diffuse anterior and posterior scleritis associated with intense circumferential perilimbal chemosis and ipsilateral yellow-white choroidal dots. A new conjunctival biopsy was performed because of unresponsiveness to high-dose systemic steroid and cyclophosphamide therapy. Immunostains for lymphocyte markers were preformed.

A morphologically and immunohistochemically typical, monotypical mu-kappa 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 remained unchanged.

When scleritis, even histologically proven, fails to respond to immunosuppressive therapy, a new biopsy is mandatory to rule out a misdiagnosed MALT lymphoma.