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Hypomelanosis of Ito

Definition

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

Alternative Names

Incontinentia pigmenti achromians

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)
  • Hearing problems
  • Increased body hair (hirsutism)
  • Scoliosis
  • Seizures
  • Streaked, whirled or mottled patches of skin on the arms, legs, and middle of the body
  • Intellectual disability including autism spectrum and learning disability
  • 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.

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
  • 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.


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.
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