Bilateral macular lesions in incontinently 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 been reported occasionally in patients with IP. These have not been symmetric, and they have not involved the fovea. The visual function in affected areas has not been described. Methods: The ophthalmic examination and results of fluorescein angiography are described in a patient with IP and bilateral discrete hypopigmented macular lesions. Results: The patient had normal vision. Fluorescein angiography of the macular lesions revealed transmission of fluorescence in the early phase and staining in the late phase. Conclusion: The authors believe that this is the first report of bilateral macular hypopig-mented lesions in a patient with IP, and it is the first to document normal vision in an affected area.