Please be patient! It may take up to ONE minute to load all the Engines.
Problems? Please contact our support.
Incontinentia pigmenti syndrome
Alternative namesBloch-Sulzberger's disease
Incontinentia pigmenti syndrome is an inherited disorder that causes unusual blistering of the skin followed by increased pigmentation.
Causes, incidence, and risk factors
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.
Signs and tests
TreatmentThere is no one treatment for IPS. Therapies must address the specific problems that are present.
Prognosis varies depending on the degree of central nervous system involvement and visual impairment.
Calling your health care providerCall 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.
PreventionGenetic counseling may be of value for prospective parents with a family history of IPS.
Update Date: 10/27/2003Jeffrey Drayer, M.D., Dermatologist, Los Angeles, CA. Review provided by VeriMed Healthcare Network.
Last updated: Tue, 06 Jan 2009 00:20:03 GMT