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Incontinentia Pigmenti

Background

Incontinentia Pigmenti: Bloch-Sulzberger syndrome

Incontinentia pigmenti (IP) is a rare inherited ectodermal dysplasia affecting the skin, hair, teeth and central nervous system. The disorder is almost always lethal in males due to miscarriage or stillbirth. This results in IP affecting females almost exclusively. IP is found in all populations but is more common in caucasian people.

What are the symptoms? View What are the symptoms?

Medical text written October 2005 by Contact a Family. Approved October 2005 by Dr H Stewart, Consultant Clinical Geneticist and Lead Clinician, Churchill Hospital, Oxford, UK. Additional ophthalmic information written March 2006 by Mr CE Willoughby, Consultant Ophthalmic Surgeon, Royal Victoria Hospital, Belfast, UK and Senior Lecturer in Ophthalmology, Queen's University, Belfast, UK.

 

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