Incontinentia pigmenti (IP) is an inherited disorder of skin pigmentation. It is associated with abnormalities of the teeth, skeletal system, eyes and central nervous system.
The is belived to be caused by mutations in a gene called NEMO (NF-kappaB essential modulator). Males are more severely affected than females. Discolored skin is caused by excessive deposits of melanin (normal skin pigment). It is one of a group of gene-linked diseases known as neurocutaneous disorders.
Most newborns with the condition will have discolored skin within the first two weeks. The pigmentation involves the trunk and extremities, is slate-grey, blue or brown, and is distributed in irregular marbled or wavy lines. The discoloration fades with age on its own and doesn't need any specific treatment.
The other symptoms of incontinentia pigmenti are as follows.
Neurological problems are associated with the condition. There could be a loss of brain tissue (known as cerebral atrophy), along with the formation of small cavities in the central white matter of the brain and the loss of neurons in the cerebellar cortex.
Some children with IP may have slow motor development. Neurological problems may cause muscle weakness in one or both sides of the body and seizures.
Chidlren with the disorder are at greater risk of visual problems, including crossed eyes, cataracts, and severe visual loss.
Dental problems are also common, particluarly missing and peg-shaped teeth.
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