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 slightly more common in girls than in boys.

Symptoms

• Crossed eyes (strabismus)
• 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 retardation

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.

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
• Mental retardation
• 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.

References

Genodermatoses and Congenital Anomolies. In: James WD, Berger TG, Elston DM, eds. Andrews' Diseases of the Skin: Clinical Dermatology. 10th ed. Philadelphia, Pa: Saunders Elsevier; 2005: 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.