PMID: 16508434Mar 2, 2006Paper

Multifocal hypopigmented retinal pigment epithelial lesions in incontinentia pigmenti

Retina
Carol L ShieldsJerry A Shields

Abstract

Incontinentia pigmenti (IP) can manifest with retinal detachment in children and can simulate retinoblastoma. The authors report a clinicopathologic correlation of IP and illustrate the retinal pigment epithelium (RPE) alterations that may be useful in establishing the diagnosis. A 30-month-old reportedly healthy girl developed strabismus and was discovered to have total retinal detachment in the left eye. Intraocular calcification was found on ultrasonography and computed tomography, suggestive of retinoblastoma. Ophthalmoscopy of the right eye revealed sector distribution of approximately 70 elongated, linear hypopigmented lesions of the RPE located in the peripheral superotemporal, inferotemporal, and superonasal quadrants and measuring 300 microm or less in basal dimension. The left eye had a total retinal detachment, vitreous traction, perivascular exudation, and iris neovascularization. Despite the lack of medical history in this patient, skin examination revealed classic cutaneous findings of IP The enucleated left globe displayed tractional retinal detachment with vitreoretinal neovascularization and nonspecific RPE papillary proliferation. Scattered minor foci of intraretinal calcification were observed. There was no s...Continue Reading

References

Apr 1, 1975·American Journal of Ophthalmology·O Mensheha-ManhartR P Mirabelli
Apr 1, 1976·Archives of Dermatology·R G Carney
Aug 15, 1992·American Journal of Ophthalmology·J A ShieldsC H Douglas
Apr 1, 1985·Ophthalmology·S I Rosenfeld, M E Smith
Dec 15, 1988·American Journal of Ophthalmology·J Pe'er
Jan 1, 1981·Ophthalmologica. Journal international d'ophtalmologie. International journal of ophthalmology. Zeitschrift für Augenheilkunde·A BerbichM Majbar
Jan 1, 1993·Journal of Medical Genetics·S J Landy, D Donnai
May 1, 1993·Archives of Ophthalmology·K J WaldT Hirose
Jan 1, 1996·Retina·M L Levy, R A Lewis
Jul 13, 2000·Acta Ophthalmologica Scandinavica·G Holmström, K Thorén
Jul 26, 2002·Journal of the American Academy of Dermatology·Alexander L BerlinLawrence S Chan
Sep 17, 2003·Archives of Dermatology·Smaïl Hadj-RabiaChristine Bodemer
Apr 1, 1952·The British Journal of Dermatology·H HABER
Jun 24, 2004·Archives of Dermatology·Morton F Goldberg

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Citations

Jul 1, 2011·Retinal Cases & Brief Reports·Pradeep S PrasadJean Pierre Hubschman
Nov 3, 2007·Ophthalmology·W Robert BellMorton F Goldberg
Sep 22, 2017·International Journal of Retina and Vitreous·Stephanie J WeissCarol L Shields

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