Navigate Up

Pediatric Center - A-Z Index

#
Q
Z

Print This Page

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.

Alternative Names

Hypomelanosis of Ito (more commonly used)

Causes

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 patches of skin on the arms, legs, and middle of the body
  • Varying degrees of intellectual disability incluiding autism and learning disability 

Exams 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.

Outlook (Prognosis)

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

Possible Complications

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

When to Contact a Medical Professional

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.

Updated: 5/15/2013

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.


©  UPMC | Affiliated with the University of Pittsburgh Schools of the Health Sciences
Supplemental content provided by A.D.A.M. Health Solutions. All rights reserved.

For help in finding a doctor or health service that suits your needs, call the UPMC Referral Service at 412-647-UPMC (8762) or 1-800-533-UPMC (8762). Select option 1.

UPMC is an equal opportunity employer. UPMC policy prohibits discrimination or harassment on the basis of race, color, religion, ancestry, national origin, age, sex, genetics, sexual orientation, marital status, familial status, disability, veteran status, or any other legally protected group status. Further, UPMC will continue to support and promote equal employment opportunity, human dignity, and racial, ethnic, and cultural diversity. This policy applies to admissions, employment, and access to and treatment in UPMC programs and activities. This commitment is made by UPMC in accordance with federal, state, and/or local laws and regulations.

Medical information made available on UPMC.com is not intended to be used as a substitute for professional medical advice, diagnosis, or treatment. You should not rely entirely on this information for your health care needs. Ask your own doctor or health care provider any specific medical questions that you have. Further, UPMC.com is not a tool to be used in the case of an emergency. If an emergency arises, you should seek appropriate emergency medical services.

For UPMC Mercy Patients: As a Catholic hospital, UPMC Mercy abides by the Ethical and Religious Directives for Catholic Health Care Services, as determined by the United States Conference of Catholic Bishops. As such, UPMC Mercy neither endorses nor provides medical practices and/or procedures that contradict the moral teachings of the Roman Catholic Church.

© UPMC
Pittsburgh, PA, USA UPMC.com