INCONTINENTIA PIGMENTI
Incontinentia Pigmenti (IP) is a genetic
disease of the skin, hair, teeth and central nervous
system. The condition was named because of the way the skin
looks under the microscope. IP was initially described in
1906 by Garrod, but the first complete description was
written by Bloch and Sulzberger in 1928. This discussion
covers what is currently known or suspected about the
disease but is not conclusive or complete. This information
was taken in part from an article by Landy and Donnai,
entitled "Incontinentia pigmenti (Bloch-Sulzberger
syndrome)" in the Journal of Medical Genetics, 1993, pages
53-59. Medical vocabulary often can be confusing and
misleading, so we recommend that parents and patients may
wish to acquire a standard medical dictionary. These are
available in the reference sections of most large
bookstores. In general, medical writing tends to be as
inclusive as possible. Therefore, descriptions of diseases
or prognoses are frequently stated as "worst-case "
situations. Remember that every sign, symptom, or finding
is not found in every patient. Parents and patients should
be selective and should interpret the information in light
of their own situation(s).
A bibliography
of articles written for
medical journals about IP is maintained by the
Foundation. Articles may be requested from the
Foundation at no charge.
Sites maintained by other groups also provide information
about IP:
Online Mendelian Inheritance in
Man
US National Institute of Neurological
Disorders and Stroke
US National Library of Medicine Genetics
Home Reference
US National Library of Medicine Medline
Plus
Gene Reviews
Gene Tests
University of Wisconsin
Healthlink
eMedicine