Incontinentia pigmenti achromians is a rare birth defect that causes unusual patches of light-colored (hypopigmented) skin and possible neurological and skeletal problems.
The cause is unknown. It is twice as common in girls than in boys.
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.
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.
What happens depends on the type and severity of symptoms that develop. In most cases, the skin pigment eventually returns to normal.
Call your health care provider if your child exhibits an unusual pattern of the color of the skin.
Hypomelanosis of Ito (more commonly used)
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 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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