Unilateral Retinopathy from Incontinentia Pigmenti
We present ultrawidefield imaging of unilateral retinal findings in a 54-year-old woman with molecularly confirmed incontinentia pigmenti and highlight extreme X-inactivation that may occur in this condition. Skin findings depicted atrophic, hypopigmented, bilateral forearm lesions (A, arrow). The left eye demonstrated widespread retinal pigmentary changes and temporal avascularity (B). Angiography confirmed temporal nonperfusion (D). The right eye was spared with a normal fundus examination, confirmed with angiography (C).