Welcome to the Incontinentia Pigmenti (IP) section
Incontinentia Pigmenti (IP) also known as Bloch-Sulzberger syndrome is a genetic condition involving a mutation in a gene (NEMO) on the X chromosome. It affects ectodermal structures (hair, skin, nails and teeth) and also the eyes and nervous system.
As IP affects the ectodermal structures the Ectodermal Dysplasia Society recognise IP as a form of ectodermal dysplasia and therefore support individuals and families with this condition. In some countries there are separate IP support groups and societies, but in the UK the Ectodermal Dysplasia Society is the recognised support group for IP.
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What is Incontinentia Pigmenti
The ectodermal dysplasias (EDs) are a group of inherited disorders that involve defects of the hair, nails, teeth and sweat glands. Depending on the particular syndrome ectodermal dysplasia (ED) can also affect the skin, the lens or retina of the eye, parts of the inner ear and other parts of the body.
Incontinentia Pigmenti is an ectodermal dysplasia and is a rare genetic disorder characterised by abnormalities of the skin, hair, teeth, eyes and nails and may be linked with neurological problems in some cases. The most characteristic and diagnostic feature of this condition are skin problems which can be described in five distinct stages:-
Vesicular - blistered skin rash, usually present at birth or soon after birth and disappearing by the age of 4 months.
Verrucous - wart-like patches on the lower limbs, usually occurs after the first stage and generally clearing up by 6 months of age.
Hyperpigmentation - dark brown streaks, typically on the skin of the trunk and groin, usually between 6 and 12 months of age. These brown streaks generally fade after a few years.
Hypopigmented - streaks, usually on the legs.
IP largely affects girls and only in exceptional circumstances does it affect boys.
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