Doctor's Notes on Incontinentia Pigmenti
Incontinentia pigmenti (IP or Bloch-Sulzberger syndrome) is an unusual inherited disorder of skin pigmentation that is associated with abnormalities of the skin, teeth, bones, brain, spinal cord and eyes. Signs and symptoms may occur first at birth with a characteristic rash that may blister; most newborns are diagnosed at birth. Skin may develop grey or brown patches. About 33% of girls will have a turned eye; others may have other abnormalities like retinal disorders, seizures, dental and bone abnormalities and intellectual disability. Most males with this disease do not survive childbirth.
The cause of Incontinentia pigmenti is genetic; it is X-linked dominantly inherited disease that is usually lethal in males. Females survive birth due to the presence of two X chromosomes where the undamaged one tries to compensate for the other damaged X chromosome.
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Kasper, D.L., et al., eds. Harrison's Principles of Internal Medicine, 19th Ed. United States: McGraw-Hill Education, 2015.