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Report From the International Conference on Incontinentia Pigmenti: Translating Discovery to Therapy
Journal article   Peer reviewed

Report From the International Conference on Incontinentia Pigmenti: Translating Discovery to Therapy

Reid Wilson, Nicole Somani, Nancy Arias, Audina Berrocal, Connie Chen, Xi Chen, Emily Cole, Phillip Ehrich, Teresa Christina Faupel, Philip Ferrone, …
American journal of medical genetics. Part A
05/11/2026
DOI: 10.1002/ajmg.a.70195
PMID: 42109079

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Abstract

Incontinentia pigmenti (IP) is a rare, X‐linked dominant condition characterized by a perinatal rash as well as abnormalities of the central nervous system (CNS), eyes, hair, nails, and teeth. The CNS and ophthalmic manifestations are potentially severe and may lead to neurologic impairment and vision loss. Due to the rarity of IP, there remains a limited understanding of its physiology, and the guidelines for screening, clinical staging, and management of complications remain controversial. To address these gaps, a multidisciplinary, international conference on IP was held in Charlotte, North Carolina, from February 21 to 23, 2025. The meeting was hosted by the National Foundation for Ectodermal Dysplasias and the Oregon Health Sciences University. This report summarizes the discussion and recommendations from the conference, including guidance for screening of ophthalmic complications, as well as next steps for pursuing therapeutic interventions.
dermatology ectodermal dysplasia genetics incontinentia pigmenti neovascularization ophthalmology retina

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