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Incontinentia pigmenti

• Overview
• Symptoms
• Treatment
• Prevention

Definition:

Incontinentia pigmenti is skin condition passed down through families that causes unusual blistering and changes in skin color.

See also: Incontinentia pigmenti achromians

Alternative Names:

Bloch-Sulzberger syndrome

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.

• Review Date: 4/16/2007
• Reviewed By: Michael S. Lehrer, M.D., Department of Dermatology, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network.

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