Fig. 1

Clinical features of incontinentia pigmenti. A 2-month-old Asian Indian female was found at 6 weeks of age to have leukocoria of the right eye (a) with a normal appearing left eye (b). Fundus examination of the right eye revealed a tractional retinal detachment (c) behind the lens, dragging the pars plicata inward and producing vitreous hemorrhage inferiorly. The left fundus (d) was normal. Fluorescein angiography of the right eye (e) revealed marked hyperfluorescence with diffuse leakage suggestive of neovascularization and the left eye (f) was healthy with normal perfusion. (g) Involuting cutaneous vesicular lesions and (h) linear hyperpigmentation (lines of Blaschko) were consistent with incontinentia pigmenti