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Incontinentia pigmenti achromians

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

Causes of Incontinentia pigmenti achromians

The cause is unknown. It is twice as common in girls than in boys.

Incontinentia pigmenti achromians Symptoms

  • Crossed eyes (strabismus)
  • Hearing problems
  • 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 intellectual disability incluiding autism and learning disability 

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.

Treatment of Incontinentia pigmenti achromians

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.

Prognosis (Outlook)

What happens depends on the type and severity of symptoms that develop. In most cases, the skin pigment eventually returns to normal.

  • Discomfort and walking problems due to scoliosis
  • Emotional distress related to the physical appearance
  • Intellectual disability
  • Seizures and resulting possible injury

Call your health care provider if your child exhibits an unusual pattern of the color of the skin.

References

James WD, Berger TG, Elston DM, Andrews' Diseases of the Skin: Clinical Dermatology. 11th ed. Philadelphia, Pa: Elsevier Saunders; 2011: chap 27.

Moss C. Mosaicism and linear lesions. In: Bolognia JL, Jorizzo JL, Schaffer JV, eds.: Dermatology. 3rd ed. Philadelphia, Pa: Elsevier Saunders; 2012:chap 61.

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Review Date: 5/15/2013
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, Bethanne Black, and the A.D.A.M. Editorial team.
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