Incontinentia Pigmenti (Bloch-Sulzberger Syndrome)

Four cases of incontinentia pigmenti, all born within a six month span in one city, are presented. This condition is probably more common than reported: 309 cases appear in the world literature, and as many cases are either overlooked, misdiagnosed, or unreported unless associated with extra-dermatological findings. Incontinentia pigmenti has been reported to be possibly hereditary, congenital, or due to viral infection or allergy. Pediatricians are often the first to see this rare condition and should be familiar with the syndrome because of possible associated abnormalities.