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Incontinentia pigmenti - side
Incontinentia pigmenti - side


Incontinentia pigmenti achromians

Definition:

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

See also: Hypopigmentation



Alternative Names:

Hypomelanosis of Ito (more commonly used)



Causes, incidence, and risk factors:

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



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


Signs and tests:

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:

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.



Support Groups:



Expectations (prognosis):

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



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


Calling your health care provider:

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



Prevention:



References:

Genodermatoses and Congenital Anomalies. In: James WD, Berger TG, Elston DM, eds. Andrews' Diseases of the Skin: Clinical Dermatology. 101th ed. Philadelphia, Pa: Saunders Elsevier; 2011: chap 27.

Moss C. Mosaicism and Linear Lesions. In: Bolognia JL, Jorizzo JL, Rapini RP, eds.: Dermatology. 2nd ed. Philadelphia, Pa: Mosby Elsevier; 2008: chap 61.




Review Date: 5/13/2011
Reviewed By: Kevin Berman, MD, PhD, Atlanta Center for Dermatologic Disease, Atlanta, GA. Review provided by VeriMed Healthcare Network; Linda Vorvick, MD, Medical Director, MEDEX Northwest Division of Physician Assistant Studies, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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