Intravitreal injection of ranibizumab in severe retinopathy of incontinentia pigmenti.

Incontinentia pigmenti (IP) is a rare X-linked multisystem disorder that mainly affects females and is usually lethal to males in utero. Mutations in the NEMO gene on chromosome Xq28 (IKBKG, inhibitor of kB kinase gamma) have been shown to cause IP. Approximately 35% of patients have ophthalmic manifestations, and 20% develop vision-threatening diseases. Retinal vascular abnormalities, from peripheral retinal avascularity to neovascular proliferation, have been described in IP patients. Laser photocoagulation and cryotherapy are traditionally effective therapy for retinopathy of IP. However, some cases still progress to retinal detachment. We report 2 cases of IP infants treated with intravitreal ranibizumab with subsequent resolution of the vasculopathy.