Incontinentia pigmenti achromians (patient information)

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

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

What are the symptoms of Incontinentia pigmenti achromians?

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

What causes Incontinentia pigmenti achromians?

The cause is unknown.

Who is at highest risk?

It is slightly more common in girls than in boys.

Diagnosis

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.

When to seek urgent medical care?

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

Treatment options

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.

Where to find medical care for Incontinentia pigmenti achromians?

Directions to Hospitals Treating Incontinentia pigmenti achromians

What to expect (Outlook/Prognosis)?

What happens depends on the type and severity of symptoms that develop. In most cases, the skin pigment eventually returns to normal.

Possible complications

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

Sources

http://www.nlm.nih.gov/medlineplus/ency/article/001461.htm

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