How is Incontinentia Pigmenti treated?
There is no specific treatment for incontinentia pigmenti. The main goal is to prevent secondary bacterial infection of skin lesions and to monitor closely the development of related problems. This should include regular dental care and close monitoring by an ophthalmologist for the first few years of life.
How common is Incontinentia Pigmenti?
Incontinentia pigmenti is an uncommon disorder. Between 900 and 1,200 affected individuals have been reported in the scientific literature. Most of these individuals are female, but several dozen males with incontinentia pigmenti have also been identified.
What is an IP diagnosis?
Summary. Incontinentia pigmenti (IP) is a genetic ectodermal dysplasia affecting the skin, hair, teeth, microvasculature, and central nervous system. Progressive skin changes occur in four stages, the first of which appear in early infancy or can be present at birth.
What is Bloch-Sulzberger syndrome?
Incontinentia pigmenti or Bloch-Sulzberger syndrome is a rare genodermatosis, linked to X chromosome, of autosomal dominant character, which affects ectodermal and mesodermal tissues, such as skin, eyes, teeth and central nervous system.
What are the effects of Incontinentia Pigmenti?
Incontinentia pigmenti (IP) is a genetic condition that affects the skin and other body systems. Skin symptoms change with time and begin with a blistering rash in infancy, followed by wart-like skin growths. The growths become swirled grey or brown patches in childhood, and then swirled light patches in adulthood.
What is Incontinentia Pigmenti disorder?
Incontinentia pigmenti (IP) is a genetic disorder with distinctive skin rashes and lesions seen at birth or within the first few weeks. The majority of children with IP don’t have complications and may be only mildly affected, if at all. But around 20% develop neurological problems that can range from mild to severe.
What are the symptoms of Incontinentia Pigmenti?
Is Incontinentia Pigmenti hereditary?
Incontinentia pigmenti (IP) is an X-linked dominant inherited (genetic) disorder. It is one of a group of neurocutaneous disorders. These types of disorders can affect the central nervous system, skin, eyes, teeth and skeletal system.
How many cases of Incontinentia Pigmenti in the world?
IP is a rare X-linked dominant disorder. About 700 to 1000 cases have been reported worldwide (about 1 in 50,000 live births); white infants are most commonly affected. In a review of 653 patients, more than half had a family history of the condition.
How many people have Hypomelanosis of Ito?
Affected Populations The incidence of hypomelanosis of Ito is estimated to be 1 in 8,000-10,000 people in the general population. The symptoms usually become apparent during the first or second year of life. Hypomelanosis of Ito was first described in the medical literature in 1952.