Abstract
PURPOSE: To describe a patient with incontinentia pigmenti (IP) and Raynaud's phenomenon (RP). OBSERVATIONS: A 5 year-old girl with history of IP was noted to have RP. Visual acuity was unaffected in both eyes, and fundus examination demonstrated regressed peripheral neovascularization. Photos of the patient's hands demonstrated pale discoloration associated with exposure to cold. CONCLUSIONS AND IMPORTANCE: IP, known to affect small cerebral and retinal blood vessels, can also affect the small blood vessels in the extremities, resulting in secondary RP.