Health Dictionary
Reliable, easy-to-read patient information on common medical conditions — written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.
Incontinentia pigmenti achromians
Introduction
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
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Want to know more?
A more detailed clinical article for the same condition is available from WikiDoc. It is written for medical professionals and uses technical language.