The Importance of Screening for Sight‐Threatening Retinopathy in Incontinentia Pigmenti

• Published in: Pediatric dermatology Vol. 21; no. 3; pp. 242 - 245
• Main Authors: Wong, Gavin A. E., Willoughby, Colin E., Parslew, Richard, Kaye, Stephen B.
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Science Inc 01.05.2004; Blackwell
• Subjects: Biological and medical sciences; Child; Child, Preschool; Dermatology; Diagnosis, Differential; Facial bones, jaws, teeth, parodontium: diseases, semeiology; Humans; Incontinentia Pigmenti - complications; Incontinentia Pigmenti - diagnosis; Male; Medical sciences; Non tumoral diseases; Ophthalmology; Otorhinolaryngology. Stomatology; Pigmentary diseases of the skin; Retinal Diseases - diagnosis; Retinal Diseases - etiology; Retinopathies; Skin - pathology; Human; Pediatrics; Skin disease; Nervous system diseases; Pigmentation disorder; Incontinentia pigmenti; Retinopathy; Stomatology; Dermatology; Dental disease; Medical screening; Genetic disease; Case study; Eye disease; Child
• ISSN: 0736-8046; 1525-1470
• DOI: 10.1111/j.0736-8046.2004.21311.x

:  Incontinentia pigmenti (IP) is an X‐linked dominant disorder of ectodermal structures affecting the skin, hair, teeth, eyes, and central nervous system. The four classic cutaneous stages of the disorder are well known to pediatric dermatologists. However, ocular and neurologic sequelae represent the major morbidity in IP. The two patients reported here highlight some of the potential ocular manifestations of IP and emphasize the importance of early ophthalmologic assessment in this condition.