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Incontinentia pigmenti - Overview

Alternative Names

Bloch-Sulzberger syndrome

Definition of Incontinentia pigmenti :

Incontinentia pigmenti is skin condition passed down through families. It leads to 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.

  • Reviewed last on: 5/13/2011
  • Kevin Berman, MD, PhD, Atlanta Center for Dermatologic Disease, Atlanta, GA. Review provided by VeriMed Healthcare Network.  Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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