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Incontinentia pigmenti - Symptom

Alternative Names

Bloch-Sulzberger syndrome

Symptoms:

Infants with IP are born with streaky, blistering areas. When the areas heal, they turn into rough bumps. Eventually, these bumps go away, but leave behind darkened skin, called hyperpigmentation. After several years, the skin returns to normal. In some adults, there may be areas of lighter colored skin (hypopigmentation).

IP is associated with central nervous system problems, including:

  • Delayed development
  • Loss of movement (paralysis)
  • Mental retardation
  • Muscle spasms
  • Seizures

Persons with IP may also have abnormal teeth, hair loss, and visual problems.

Signs and tests:

The doctor will perform a physical exam, look at the eyes, and test muscle movement.

There may be unusual patterns and blisters on the skin, as well as bone abnormalities. An eye exam may reveal cataracts, strabismus (crossed eyes), or other problems.

  • Reviewed last on: 4/15/2009
  • Jonathan Kantor, MD, North Florida Dermatology Associates, Jacksonville, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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