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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.

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.

 
  • 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.

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