BILATERAL MACULAR LESIONS IN INCONTINENTIA PIGMENTI - BLOCH-SULZBERGER SYNDROME

Background: Incontinentia pigmenti (IP) is an inherited disorder with many systemic and ocular manifestations. Abnormalities of the retinal pigment epithelium, including peripheral hypopigmented areas, have bee n reported occasionally in patients with IP. These have not been symme tric, and they have not involved the fovea. The visual function in aff ected areas has not been described. Methods: The ophthalmic examinatio n and results of fluorescein angiography are described in a patient wi th IP and bilateral discrete hypopigmented macular lesions. Results: T he patient had normal vision. Fluorescein angiography of the macular l esions revealed transmission of fluorescence in the early phase and st aining in the late phase. Conclusion: The authors believe that this is the first report of bilateral macular hypopigmented lesions in a pati ent with IF, and it is the first to document normal vision in an affec ted area.