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As there is no cure for incontinentia pigmenti, treatment is based on symptoms. The risk of infection from blisters is a consideration, and topical medications can often be used to lessen pain
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What is incontinentia pigmenti?
Incontinentia pigmenti is a rare genetic disease resulting in a neurocutaneous disorder affecting the nervous system whose abnormalities can involve the skin, hair, and teeth of affected individuals.
What types of professionals assist in treatment of incontinentia pigmenti?
The treatment team consists of a neurologist , clinical geneticist, genetic counselor, speech pathologist, ophthalmologist, and a dermatologist.
What is the average life span of a person with incontinentia pigment?
My daughter was born with Incontinentia Pigmenti and she will be celebrating her 50th birthday in November
What neurological problems are associated with incontinentia pigmenti?
Neurological problems associated with incontinentia pigmenti include cerebral atrophy, leading to poor muscle control and weakness. Mental retardation and seizures are also similarly present.
How is diagnosis for incontinentia pigmenti conducted?
Diagnosis is achieved first by a clinical diagnosis from a clinical geneticist, followed by molecular genetic testing in a CLIA-approved diagnostic laboratory
What are the stages in which incontinentia pigmenti produces defects in the skin?
The first stage usually occurs before four months old when the blisters appear in the skin. The second stage involves a wart-like rash, while the third brings darkened pigmentation and the last,linear hypopigmentation
What role does heredity play in transmission of incontinentia pigmenti?
The gene that is defective in this disease is located on the X chromosome and is inherited as a dominant disorder, meaning that a child of an affected mother has a 50% risk of inheriting the disorder. If mother has IP then female child has 50% chance of inheriting IP. The male child with IP usually do not survive.
Incontinentia pigmenti?
DefinitionIncontinentia pigmenti is skin condition passed down through families. It leads to unusual blistering and changes in skin color.See also: Incontinentia pigmenti achromiansAlternative NamesBloch-Sulzberger syndromeCauses, incidence, and risk factorsIncontinentia pigmenti (IP) is caused by a genetic defect. In most cases, there is a problem with one of the genes located on the X chromosome.See also: X-linked traitThe condition is most often seen in females. When it occurs in males, it can be deadly.Most babies born with IP develop discolored skin within the first 2 weeks. The discolored skin occurs when a substance called melanin builds up under the skin. Melanin gives skin its color.SymptomsInfants with IP are born with streaky, blistering areas. When the areas heal, they turn into rough bumps. Eventually, these bumps go away, but leave behind darkened skin, called hyperpigmentation. After several years, the skin returns to normal. In some adults, there may be areas of lighter colored skin (hypopigmentation).IP is associated with central nervous systemproblems, including:Delayed developmentLoss of movement (paralysis)Mental retardationMuscle spasmsSeizuresPersons with IP may also have abnormal teeth, hair loss, and visual problems.Signs and testsThe doctor will perform a physical exam, look at the eyes, and test muscle movement.There may be unusual patterns and blisters on the skin, as well as bone abnormalities. An eye exam may reveal cataracts, strabismus (crossed eyes), or other problems.TreatmentThere is no specific treatment for IP. Treatment is aimed at the individual symptoms. For example, glasses may be needed to improve vision. Medicine may be prescribed to help control seizures or muscle spasms.Expectations (prognosis)How well a person does depends on the severity of central nervous system involvement and eye problems.ComplicationsDelayed developmentInfections of blistered skinMental retardationMuscle spasticityParalysisSeizuresWalking difficultyVision problemsCalling your health care providerCall your health care provider if:You have a family history of IP and are considering having childrenYour child has symptoms of this disorderPreventionGenetic counseling may be helpful for those with a family history of IP who are considering having children.
What could be the cause of blisters on new born baby?
Two of the main causes of blisters on a newborn baby are toxic erythema of the newborn and malaria. Other possible causes include bacteria, fungi and viral infections, neonatal cephalic pustulosis or incontentia pigmenti.
What is an adjunct treatment?
An adjunct treatment is an additional treatment used for increasing the efficacy or safety of a primary treatment.
TennisElbow Treatment?
Neck Pain Treatment, and Shoulder Pain Treatment to Neuropathy Treatment, Spinal Decompression
What is a collective noun for treatment?
A collective noun for treatment is a course of treatment.
