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

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

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

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.
The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
adam.com