Zur Kenntnis der Incontinentia pigmenti Block-Sulzberger

Corneal Abnormalities in Incontinentia Pigmenti

“…23 The most frequently reported corneal findings are secondary to other processes and include band-shaped keratopathy in a phthisical eye, 24 megalocornea caused by congenital glaucoma, 25 corneal edema in eyes associated with cataract and retinal detachment, 6 and a small, flattened cornea with iridocorneal adhesions. 3 Descriptions of primary keratopathies, similar to that in our patient, include a ''vortex epithelial keratitis,'' with radial lines of epithelial microcysts staining with fluorescein in 2 patients, with 1 having ''midstromal mild haziness.'' 26 Another report detailed ''bilateral superficial minimal nonstaining corneal irregularity.''…”

“…2 A wide range of reported ocular manifestations include complications resulting from proliferative retinal disease and congenital anomalies such as ptosis, microphthalmos, buphthalmos, iris atrophy, congenital cataract, uveitis, myopia, blue sclera, strabismus, nystagmus, retinal detachment, retrolental mass, pigmentary changes of the fundus, foveal hypoplasia, optic atrophy, retinal dysplasia, pseudoglioma, and phthisis bulbi. [3][4][5][6][7][8][9][10][11][12][13] Several cases of unusual superficial corneal abnormalities have also been described. [14][15][16] We report a patient with corneal changes and its histologic and in vivo confocal microscopic correlations.…”

Novel corneal features in two males with incontinentia pigmenti

“…Previous reports of corneal changes in IP are varied and include band shaped keratopathy in a phthisical eye with a retinal "glioma" 19 ; bilateral superficial round lesions like bullous keratopathy, involving the epithelium and the adjacent stroma 20 ; a small, flattened cornea with corneal flecks at sites of iridocorneal attachments with an irregularly broadened and opacified limbus 21 ; megalocornea secondary to congenital glaucoma resulting from an iris cyst 22 ; "bilateral superficial minimal non-staining corneal irregularity" 15 ; corneal oedema associated with cataract 23 ; and epithelial disturbance (staining with fluorescein) with "mid-stromal mild haziness." 24 Histological studies of eyes enucleated in the presence of a retrolental mass report no corneal abnormalities.…”

Dominant exudative vitreoretinopathy and other vascular developmental disorders of the peripheral retina

“…Disease symptoms have also been observed in the anterior segment: posterior synechiae were described by Uebel et al (1950), Scott et al (1955), Wollensak (1959) and Mensheha-Manhart et al (1975).…”