Alternative names
Incontinentia pigmenti achromians

Definition

Incontinentia pigmenti achromians is a congenital 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 (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 physician may also recommend chromosome analysis or a further medical workup to discover any associated 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 subsequent injury  
• discomfort and gait problems related to scoliosis  
• emotional distress related to the cosmetic effects

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

Medical Encyclopedia

All ArmMed Media material is provided for information only and is neither advice nor a substitute for proper medical care. Consult a qualified healthcare professional who understands your particular history for individual concerns.