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Incontinentia pigmenti on the leg
Incontinentia pigmenti on the leg


Incontinentia pigmenti on the leg
Incontinentia pigmenti on the leg


Definition:

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



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.



Treatment:

There is no specific treatment for IP. Treatment is aimed at the individual symptoms. For example, glasses may be needed to improve vision. Medicine may be prescribed to help control seizures or muscle spasms.



Support Groups:



Expectations (prognosis):

How well a person does depends on the severity of central nervous system involvement and eye problems.



Complications:

Calling your health care provider:

Call your health care provider if:

  • You have a family history of IP and are considering having children
  • Your child has symptoms of this disorder


Prevention:

Genetic counseling may be helpful for those with a family history of IP who are considering having children.




Review Date: 4/15/2009
Reviewed By: 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.

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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