Corneal perforation due to limbal involvement in Sézary syndrome

Abstract

The majority of lymphomas involving the eye and ocular adnexa are B-cell lymphomas. Ocular involvement by T-cell lymphoma is rare. We report a case of corneal perforation due to direct involvement of the corneal limbus by lymphoma in a patient with Sézary syndrome. A 58-year-old male with cutaneous T-cell lymphoma presented with painful, left-sided corneal ulceration, a dense infiltrate, severe epitheliopathy, hypopyon and a diffuse confluent dermatitis involving the lids. He had a history of Sézary syndrome. Despite maximal treatment, this severe ulcerative keratitis progressed to central corneal perforation. The eye was subsequently enucleated and submitted for histopathological examination. Histopathological examination confirmed corneal ulceration with perforation. There was an infiltrate of large atypical cells at the limbus, with tropism for the overlying epithelium. Immunohistochemical staining of these cells was positive for CD2, CD3, CD5, CD4 and CD7. Staining for CD8, CD30 and CD56 was negative. The appearances were those of an epitheliotrophic T-cell lymphoma, and were considered to represent spread from the patient's underlying Sézary syndrome. The patient died 2months later from bronchopneumonia. Ocular involvement by cutaneous T-cell lymphoma usually occurs in advanced disease, and carries a poor prognosis. This patient was immunocompromised due to advanced malignancy, and there was a high suspicion of infection as the primary cause of corneal ulceration. This case highlights that it is important to consider direct tumour infiltration as an initiating or contributing factor for corneal ulceration in such patients.