Incontinentia pigmenti is skin condition passed down through families. It leads to unusual blistering and changes in skin color.

See also: Incontinentia pigmenti achromians

Bloch-Sulzberger syndrome

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.

Images

Incontinentia pigmenti on the leg

Incontinentia pigmenti on the leg

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Incontinentia pigmenti Symptoms

Review Date: 05/13/2011
Reviewed By: 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.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org)