Incontinentia Pigmenti Syndrome
Alternate Names : Bloch-Sulzberger's Disease
Definition
Incontinentia pigmenti syndrome is an inherited disorder that causes unusual blistering of the skin followed by increased pigmentation.
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Overview, Causes, & 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.
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