Incontinentia pigmenti achromians

Definition

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

Causes, incidence, and risk factors

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

Symptoms

• Streaked, whirled or mottled areas of hypopigmentation  on limbs and trunk
• 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.

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.

Illustrations

Incontinentia pigmenti - side

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Hypomelanosis of Ito ; hypomelanosis of ito