Candida retinitis in bare lymphocyte syndrome

Bare lymphocyte syndrome (BLS) is a rare, severe combined immunodeficiency characterized by lack of expression of HLA A, B and C antigens and the absence of B2 microglobulins. Patients with BLS exhibit functional deficiency of both T and B cells resulting in bacterial as well as viral and fungal infection. Ophthalmic findings in this group of disorders have not been reported. We present a case of candida retinitis in a terminally ill 5-year-old girl with BLS.