Incontinentia Pigmenti
By Pragat Muthu
Incontinentia pigmenti
Incontinentia pigmenti is a disorder that affects the skin, hair, teeth, nails, and nervous system. This disease was discovered Bruno Bloch in 1926 in Germany and discovered again by Marion Sulzberger in 1928 in USA. Incontinentia pigmenti is inherited through x-linked dominant genes and is located at the chromosomal locus Xp11. It is an uncommon disease. There have only been 900 and 1,200 noted incidences of IP. They are mainly female, however there are still some males with the disease.
Symptoms
- Alopecia(baldness)
- hypodontia(tooth loss),
- abnormal tooth shape
- mental retardation
- discoloration
- Cerebral atrophy (cavities in brain)
- Seizures
- Muscle spasms
- paralysis
- Dwarfism
The stages of incontenia pigmenti
The victim has blisters till four months of age. The next stage includes wart like rashes for several months. The third step is swirling macular hyperpigmentation, which is splotchy pigmentation, from six months into adulthood. The fourth stage is linear hypopigmentation, or loss of pigments.
Prognosis
The skin problems usually get better and may even disappear, but there may be brain problems. Some other effects include death, learning disabilities and hyper pigmentation.
Treatment
Some things you can do to treat this is
- Waiting
- therapy
- There are a few products that may help