Incontinentia Pigmenti is a rare genetic condition. It is a dominant X-linked trait, i.e. comes from the maternal side. It is possible that there is no familial history of this condition, then it would be a spontaneous mutation. Only 3% of males are affected by this condition. Most males are miscarried. This is pretty much a female only condition.

Diagnoses of this condition is done by taking a small sample of the lesion, called a biopsy. Genetic testing may be offered to you and your child. Only one lab in the U.S.A. does such testing for this condition and it is in Texas.

This condition is caused by excessive melanin (the pigment of your skin) deposits. The rash is progressive, coming in four stages, which I have previously described. There is no treatment for the rash. The best thing is to let it run its' course and keep the areas affected clean and dry to prevent secondary infection.

The gene was recently identified in this condition. It is the NEMO gene. Two chromosomes are affected: X;p;11 and X;q;28.

Recommendations:

Most Doctor's will recommend the following course of action:

* Continue to follow up with a Dermatologist who is very familiar with this condition.
* Child's first visit to the Opthalmologist should be before 3 months.
* A Dentist should evaluate the teeth before the child is 1 year of age.
* A Neurologist should be seen if seizure activity or other nervous system disorders are suspected. Many Neuro's will require an EEG before the child is seen. Contact baby's pediatrician for a good referral.
* I highly recommend learning all you can about this to educate yourself and your family members. Your child will need to know when she gets older. I also recommend you print out everything and anything you can on this and give it to whoever treats your child on a regular basis.