Retinal tears occurring at the border of vascular and avascular retina in adult patients with incontinentia pigmenti.

Incontinentia pigmenti (IP) is an X-linked dominant disorder with characteristic cutaneous, dental, skeletal, central nervous system, and ocular manifestations; it is lethal in most male embryos.1 The precise pathogenesis of the clinical manifestations of IP is unknown. Clinical suspicion of the diagnosis is usually a result of the characteristic rash that develops at or shortly after birth and is confirmed by skin biopsy, showing intraepithelial vesicles filled with eosinophils.1 Systemic manifestations often become evident within the first 4 months of life and are seen in 80% of patients. Ocular involvement occurs in 35% of persons with IP; findings include strabismus, conjunctival pigmentation, cataracts, corneal epithelial and stromal keratitis, iris hypoplasia, optic atrophy, and, most important, vitreoretinal abnormalities.1 Among the vitreoretinal manifestations are persistence of fetal vasculature, foveal atrophy, paramacular vascular dilatations and aneurysms, retinal pigment epithelium mottling, retinal pigment epithelium hypopigmentation, peripheral retinal avascular zones and vascular tortuosity, arteriovenous anastomoses, preretinal neovascularization at the junction of vascular and avascular retina, vitreous hemorrhage, and tractional retinal detachment.1,2 We describe two adults with IP who had retinal tears at the junction of the peripheral vascularized and nonvascularized areas of the retina. Rhegmatogenous retinal detachment in adults with IP has not been previously reported, although combined tractional and rhegmatogenous retinal detachment has been described in an infant.3 Case Reports