Incontinentia pigmenti
Definition
Incontinentia pigmenti (IP) is an X-linked dominant disorder affecting primarily the skin, hair, teeth and nails (all components of the epidermis). This disease may have been initially described by Garrod in 1906. It was completely characterized by Bloch and Sulzberger in 1928. For this reason, incontinentia pigmenti has also been referred to as Bloch-Sulzberger syndrome.
Description
Incontinentia pigmenti has been traditionally classified into two types: type I and type II. Much debate has occurred over whether or not type I, or sporadic, incontinentia pigmenti is actually the same disease as type II, or familial, male-lethal type, incontinentia pigmenti. The debate on this issue continues in the medical literature in early 2001. The growing consensus is that sporadic (type I)...
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