Incontinentia Pigmenti

Incontinentia pigmenti (IP) is a rare, genetic disorder characterized by unusual patterns of discolored skin. The disorder is caused by excessive deposits of melanin (normal skin pigment).

IP is divided into 4 stages, which frequently overlap or appear together. During the first stage, which begins between birth and 6 months of age, there is inflammation accompanied by skin redness and spiral lines of small fluid-filled blisters. The second stage gradually develops with rough, warty skin growths which appear on the arms or legs and, sometimes, on the head or trunk. These growths, which are often arranged in the same spiral or linear pattern as in the first stage, usually resolve during infancy or early childhood. The third stage begins between 3 months and 2 years of age and is characterized by discolorations appearing in unusual patterns. The fourth stage consists of diminished pigmentation or atrophy in areas of previous discoloration.

In rare cases of IP, hair loss with scarring and non-dermatological symptoms such as dental problems (delayed tooth growth or decay, missing or malformed teeth), diminished vision, seizures, muscle spasms, or slight paralysis may occur. Developmental abnormalities including dwarfism or short stature, club foot, spina bifida, skull and ear deformities, cleft lip or palate, atrophy on one side of the body, abnormal development of cartilage, congenital dislocation of hip, incomplete development of one side of the spinal bones, and extra ribs or webbed fingers may occur with the disorder but are not characteristic. In a few cases of IP, extremely wooly or kinky hair and an immune system dysfunction may also appear.

The skin abnormalities of IP usually disappear by adolescence or adulthood without treatment. Diminished vision may be treated with corrective lenses, medication, or, in severe cases, surgery. Dental and hair problems may be treated by a specialist. Neurological symptoms such as seizures, muscle spasms, or mild paralysis may be controlled with medication and/or medical devices and with the advice of a neurologist.

Although the skin abnormalities usually regress, and sometimes disappear completely, there may be residual neurological difficulties.

Information provided by the
National Institute of Neurological Disorders and Stroke,
National Institutes of Health

Each year, Medical College of Wisconsin physicians care for more than 180,000 patients, representing nearly 500,000 patient visits. Medical College physicians practice at Children's Hospital of Wisconsin, Froedtert Memorial Lutheran Hospital, the Milwaukee VA Medical Center, and many other hospitals and clinics in Milwaukee and southeastern Wisconsin.