Incontinentia pigmenti achromians

Alternative Names

Hypomelanosis of Ito (more commonly used)

Definition of Incontinentia pigmenti achromians

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

Causes, incidence, and risk factors

The cause is unknown. It is slightly more common in girls than in boys.

Signs and tests

A Wood’s lamp examination of the 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)

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

Review

Kevin Berman, MD, PhD, Atlanta Center for Dermatologic Disease, Atlanta, GA. Review provided by VeriMed Healthcare Network; Linda Vorvick, MD, Medical Director, MEDEX Northwest Division of Physician Assistant Studies, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc. – 8/26/2009

Incontinentia pigmenti - side

ADAM Medical Encyclopedia

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