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Alternative names Return to top
Bloch-Sulzberger's diseaseDefinition Return to top
Incontinentia pigmenti syndrome is an inherited disorder that causes unusual blistering of the skin followed by increased pigmentation.
Causes, incidence, and risk factors Return to top
Incontinentia pigmenti syndrome (IPS) is inherited as a dominant X-linked trait. Almost all cases are among females and the condition may be lethal in males. IPS may also arise as a spontaneous mutation.
Infants with IPS are born with blistery (vesicular) lesions which appear as streaks. These lesions heal as rough or verrucous papules. Eventually, these papules clear but leave damaged hyperpigmented (too much pigment) skin behind. After several years, the skin returns to normal. In some adults, whorls and streaks of faint hypopigmentation (less pigment than normal) may appear.
Most people with IPS also have other problems including abnormal teeth, hair loss, and central nervous system (CNS) abnormalities. CNS problems may include retardation and delayed development, seizures, spasticity, and paralysis. Visual problems may develop in up to a third of affected people.
Symptoms Return to top
Signs and tests Return to top
Treatment Return to top
There is no one treatment for IPS. Therapies must address the specific problems that are present.Expectations (prognosis) Return to top
Prognosis varies depending on the degree of central nervous system involvement and visual impairment.
Complications Return to top
Calling your health care provider Return to top
Call your health care provider if you have a family history of this disorder and are considering having children, or if your child shows symptoms that characterize this disorder.Prevention Return to top
Genetic counseling may be of value for prospective parents with a family history of IPS. Update Date: 10/27/2003 Updated by: Jeffrey Drayer, M.D., Dermatologist, Los Angeles, CA. Review provided by VeriMed Healthcare Network.
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Page last updated: 28 October 2004 |