MedlinePlus Medical Encyclopedia: Incontinentia pigmenti achromians

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Incontinentia pigmenti achromians

Contents of this page:
Illustrations Alternative names Definition Causes, incidence, and risk factors Symptoms	Signs and tests Treatment Expectations (prognosis) Complications Calling your health care provider

Illustrations

Incontinentia pigmenti - side

Alternative names Return to top

Hypomelanosis of Ito

Definition Return to top

Incontinentia pigmenti achromians is a congenital disorder that causes unusual and sometimes bizarre patterns of hypopigmented (diminished pigment) skin.

Causes, incidence, and risk factors Return to top

The cause of this disorder is unknown, though most patients have abnormal chromosomes. Girls are affected slightly more often than boys.

Symptoms Return to top

streaked, whirled or mottled areas of hypopigmentation (limbs and trunk)
varying degrees of retardation
seizures
crossed eyes (strabismus)
increased body hair (hirsutism)
scoliosis

Signs and tests Return to top

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

Treatment Return to top

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.

Expectations (prognosis) Return to top

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

Complications Return to top

mental retardation
seizures and subsequent injury
discomfort and gait problems related to scoliosis
emotional distress related to the cosmetic effects

Calling your health care provider Return to top

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

Update Date: 10/27/2003

Updated by: Jeffrey Drayer, M.D., Dermatologist, Los Angeles, CA. Review provided by VeriMed Healthcare Network.

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