Hypomelanosis of Ito
Incontinentia pigmenti achromians
Hypomelanosis of Ito is a rare birth defect that causes unusual patches of light-colored ( hypopigmented ) skin and possible neurological and skeletal problems.
Hypopigmented
Skin that has turned darker or lighter than normal is usually not a sign of a serious medical condition.
Causes
Health care providers do not know the exact cause, but they believe it is a problem with genes. It is twice as common in girls than in boys.
Symptoms
Symptoms may include:
-
Crossed eyes
(strabismus)
Crossed eyes
Strabismus is a disorder in which both eyes do not line up in the same direction. Therefore, they do not look at the same object at the same time. ...
- Hearing problems
-
Increased body hair (
hirsutism
)
Hirsutism
Most of the time, women have fine hair above their lips and on their chins, chests, abdomens, or backs. The growth of coarse dark hair in these area...
-
Scoliosis
Scoliosis
Scoliosis is an abnormal curving of the spine. Your spine is your backbone. It runs straight down your back. Everyone's spine naturally curves a b...
-
Seizures
Seizures
A seizure is the physical findings or changes in behavior that occur after an episode of abnormal electrical activity in the brain. The term "seizure...
- Streaked, whirled or mottled patches of skin on the arms, legs, and middle of the body
-
Intellectual disability including
autism spectrum
and learning disability
Autism spectrum
Autism spectrum disorder (ASD) is a developmental disorder. It often appears in the first 3 years of life. ASD affects the brain's ability to devel...
- Mouth or tooth problems
Exams and Tests
A Wood's lamp examination of the skin lesions may help confirm the diagnosis. The provider may also recommend chromosome analysis or a further medical workup to discover any related medical problems.
Skin lesions
Rashes involve changes in the color, feeling or texture of your skin.
Treatment
There is no treatment for the skin patches. Cosmetics or clothing may be used to cover the patches. Seizures, scoliosis, and other problems are treated as needed.
Outlook (Prognosis)
What happens depends on the type and severity of symptoms that develop. In most cases, skin color eventually returns to normal.
Possible Complications
Problems that may result from this condition include:
- Discomfort and walking problems due to scoliosis
- Emotional distress related to the physical appearance
-
Intellectual disability
Intellectual disability
Intellectual disability is a condition diagnosed before age 18 that includes below-average intellectual function and a lack of skills necessary for d...
- Injury from seizures
When to Contact a Medical Professional
Call your provider if your child has an unusual pattern of the color of the skin.
References
Habif TP. Light-related diseases and disorders of pigmentaiton. In: Habif TP, ed. Clinical Dermatology: A Color Guide to Diagnosis and Therapy . 6th ed. Philadelphia, PA: Elsevier Mosby; 2016:chap 19.
Patterson JW. Disorders of pigmentation. In: Patterson JW, ed. Weedon's Skin Pathology . 4th ed. Philadelphia, PA: Elsevier; 2015:chap 10.
-
Incontinentia pigmenti - side - illustration
Incontinentia pigmenti causes swirling pigmentation of the skin. It is seen more frequently in females. The skin lesions are divided into three stages: blistering (with vesicles and bullae) present at birth or within 6-7 weeks, a verrucous (rough wart-like stage), and the final stage shows whorled and bizarre patterns of dark pigmentation (hyperpigmentation).
Incontinentia pigmenti - side
illustration
-
Incontinentia pigmenti - side - illustration
Incontinentia pigmenti causes swirling pigmentation of the skin. It is seen more frequently in females. The skin lesions are divided into three stages: blistering (with vesicles and bullae) present at birth or within 6-7 weeks, a verrucous (rough wart-like stage), and the final stage shows whorled and bizarre patterns of dark pigmentation (hyperpigmentation).
Incontinentia pigmenti - side
illustration
Review Date: 4/14/2015
Reviewed By: Kevin Berman, MD, PhD, Atlanta Center for Dermatologic Disease, Atlanta, GA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.