PMC5603187_01
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
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Fig1
OD, scattered posterior synechiae of the iris, and dense retrolental fibrovascular membrane with anterior and centripetal displacement of the ciliary processes (Fig. 1a, b). On anterior examination, OS appeared normal with horizontal corneal diameter of 11 mm.  There was mild hemorrhage inferiorly within the vitreous cavity (Fig. 1c). Fundus examination OS was normal without evidence of peripheral traction or retinal detachment (Fig. 1d). There was no evidence of leakage OS (Fig. 1e, f). Cutaneous examination disclosed extensive linear hyperpigmented streaks along the lines of Blashko (Fig. 1g, h).