Drexel University College of Medicine

Diseases and Conditions

Incontinentia pigmenti achromians

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

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.

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.

References

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