Incontinentia Pigmenti Overview
Incontinentia pigmenti, sometimes referred to as IP or Bloch-Sulzberger syndrome, is an unusual inherited disorder of skin pigmentation that is associated with abnormalities of the skin (100% of affected people), teeth (90% of affected people), bones (40% of affected people), brain or spinal cord (40% of affected people), and eyes (35% of affected people). The exact cause of IP is not yet known.
Incontinentia pigmenti is often diagnosed in affected people when they are newborns because of the presence of a characteristic rash. The skin changes often disappear with time, but an evaluation for nervous system and eye disturbances must be promptly initiated.
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