The Bloch-Sulzberger Syndrome (Incontinentia Pigmenti)

A Case of Incontinentia Pigmenti

“…Histopathological investigation gave the same results which were found by Haber [14], Epstein [8,9] and Grueneberg [13]. Even regard ing the developmental defects seen in our case no new points of view can be presented; symptoms in the sense of Little's disease were de scribed by other authors as well as strabismus or anomalies in the developm ent of the teeth.…”

“…Some authors [13,14,321 suggested the possibility that a presumable virus in fection of mother and fetus which occurred during the sixth month of pregnancy may be of etiologic significance in the disease. Moreover, Haber [14] supposed that the disturbance might he caused by any other infection or even by a variety of inflammatory conditions including lupus erythematosus or lichen planus in the mother.…”

“…Moreover, Haber [14] supposed that the disturbance might he caused by any other infection or even by a variety of inflammatory conditions including lupus erythematosus or lichen planus in the mother. Uebel et al [32] called attention to the fact that the histological changes in the early stage of I. p. resemble those found in allergic-hyperergic inflammatory processes of the dermis (perivascular foci of inflammation, degenerative changes in the walls of deeper arteries).…”

“…The ocular defects are: cataract, sometimes bilateral [4,12,24], blindness, retinal detachment [19], strabismus, corneal opacities [8|, blue sclerae, myopia, optic atrophy, retrolental fibroplasia [29,33], pseudo-glioma [29], nystagmus [14], exsuda tive chorioretinitis, metastatic ophthalmitis [23].…”

Ocular Findings in a Case of Incontinentia Pigmenti (Bloch-Sulzberger Syndrome)

“…35% of the patients have ocular lesions and 7 % become blind [7], Strabismus and nystagmus are often present, but they are usually secondary to another ocular or central nervous system anomaly. Cataracts, corneal opacities [6], uveitis [1,35] and blue sclerae [2,15,16,30] have also been re ported. However, the changes of the eyegrounds are especially dangerous because they usually result in a considerable loss of vision.…”