Alternative Names
Bloch-Sulzberger syndrome
Definition of Incontinentia pigmenti :
Incontinentia pigmenti is skin condition passed down through families that causes unusual blistering and changes in skin color.
See also: Incontinentia pigmenti achromians
Causes, incidence, and risk factors:
Incontinentia pigmenti (IP) is caused by a genetic defect. In most cases, there is a problem with one of the genes located on the X chromosome.
See also: X-linked trait
The condition is most often seen in females. When it occurs in males, it can be deadly.
Most babies born with IP develop discolored skin within the first 2 weeks. The discolored skin occurs when a substance called melanin builds up under the skin. Melanin gives skin its color.
