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Incontinentia pigmenti - side
Incontinentia pigmenti - side


Definition:

Incontinentia pigmenti achromians is a rare birth defect that causes unusual patches of light-colored (hypopigmented) skin and possible neurological and skeletal problems.

See also: Hypopigmentation



Alternative Names:

Hypomelanosis of Ito



Causes, incidence, and risk factors:

The cause is unknown. Girls are affected slightly more often than boys.



Symptoms:
  • Streaked, whirled or mottled patchs of skin on the arms, legs, and middle of the body
  • Varying degrees of retardation
  • Seizures
  • Crossed eyes (strabismus)
  • Increased body hair (hirsutism )
  • Scoliosis


Signs and tests:

A Wood's lamp examination of the skin lesions may help confirm the diagnosis. Your doctor may also recommend chromosome analysis or a further medical workup to discover any related medical problems.



Treatment:

There is no treatment for the hypopigmentation. Treatment consists of treating the symptoms. Cosmetics or clothing may be used to cover the hypopigmented spots if desired. Seizures, scoliosis, and other problems are treated as necessary.



Support Groups:



Expectations (prognosis):

The outcome varies depending on the type and severity of symptoms that develop. In most cases, the skin pigment eventually returns to normal.



Complications:
  • Mental retardation
  • Seizures and resulting possible injury
  • Discomfort and walking problems due to scoliosis
  • Emotional distress related to the physical appearance


Calling your health care provider:

Call your health care provider if your child exhibits an unusual pattern of the color of the skin.



Prevention:




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.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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Wentworth-Douglass Hospital
789 Central Avenue, Dover, NH 03820
Phone: (603) 742-5252
Toll free: 1 (877) 201-7100