Incontinentia Pigmenti

Fundus changes in incontinentia pigmenti (Bloch-Sulzberger syndrome): a case report.

“…Nystagmus, strabismus, myopia, microphthalmos, and phthisis bulbi have also been described. A number of cases of IP have been reported with associated posterior segment changes which include optic atrophy (Carney, 1951;Lapiere, 1951), papillitis (Kuhling, 1949), persistent hyperplastic primary vitreous, metastatic ophthalmitis (Kawamura, 1954;Scott and others, 1955). ablatio falciformis, retrolental fibroplasia with or without pseudoglioma, retinal dysplasia (Findlay, 1952;Cole and Cole, 1959;Jones, 1966;Mensheha-Manhert and others, 1975;Miller and Anderson, 1966;Zweifach, 1966).…”

Retinoblastoma in Bloch-Sulzberger Syndrome

“…How ever, pigmentary changes of the retinal pigment epithelium have also been reported, some of these being of considerable degree with occasional vascular anomalies |G arrod, 1906; Sulzberger, 1938;Carney, 1951;Findlay, 1952;Scott el al., 1955;J ensen, 1965;Cole and Cole, 1959;Jones, 1966;M iller and Anderson, 1966;Zweii ach, 1966;McC rary and Smith, 1968;F ischbein el al., 1972;Watzke et al, 1974], It is generally agreed that the Bloch-Sulzberger syndrome occurring predominantly in females is a familial disease, genetically dominant with variable penetrance. A maternal viral infection has been inculpated.…”

Incontinentia Pigmenti.

“…The initial lesion is commonly morbillifonn and infiammatory in character, as in the ease reported here. Carney (1951) has pointed out that about half the patients exhibit an infiammatory infiltration, about 30% present buUae and vesiculation as an early symptom, and in about a third of the total number of patients the lesions are warty. Early vesicular lesions were fomid to be quite common by Lewis (1954).…”