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
Hypomelanosis of ItoCauses
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
Exams and Tests
A Wood's lamp examination of the skin lesions may help confirm the diagnosis. Yourdoctor 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.
Outlook (Prognosis)
The outcome varies depending on the type and severity of symptoms that develop. In most cases, the skin pigment eventually returns to normal.
Possible Complications
- Mental retardation
- Seizures andresulting possibleinjury
- Discomfort andwalking problemsdue to scoliosis
- Emotional distress related to the physical appearance
When to Contact a Medical Professional
Call your health care provider if your child exhibits an unusual pattern of the color of the skin.
Reviewed By: Michael S. Lehrer, M.D., Department of Dermatology, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network.
