Incontinentia pigmenti with secondary Raynaud's phenomenon: A case report and review of the literature

Margaret A Greven; Darius M Moshfeghi

doi:10.1016/j.ajoc.2016.12.012

Incontinentia pigmenti with secondary Raynaud's phenomenon: A case report and review of the literature

Am J Ophthalmol Case Rep. 2016 Dec 26:6:27-29. doi: 10.1016/j.ajoc.2016.12.012. eCollection 2017 Jun.

Authors

Margaret A Greven¹, Darius M Moshfeghi¹

Affiliation

¹ Byers Eye Institute, Horngren Family Vitreoretinal Center, Dept. of Ophthalmology, Stanford University School of Medicine, Palo Alto, CA, United States.

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.

Keywords: Genetics; Incontinentia pigmenti; Pediatric ophthalmology; Retina.

Publication types

Case Reports