Abstract
Incontinentia pigmenti (IP) is an X-linked dominant disorder characterized by abnormalities of ectodermal tissues including skin, eye, tooth and hair as well as neurological deficiencies. The occurence of IP is approximately 1:40,000 in girls and is more prevelant in Caucasians. This article would present a case with proved Incontinentia pigmenti, whose dental manifestations included hypodontia, delayed resorption of primary teeth, delayed eruption of permanent teeth, and notch-shaped incisors. The dental conditions included anterior crossbite with functional shift, upper arch constriction, skeletal class Ⅲ tendency and deep bite. The Hyrax and face-mask were applied in the phase Ⅰ treatment. The treatment results showed correction of anterior crossbite, increasing of mandibular plane angle and expansion of upper arch width. The further orthodontic treatment will be given after all the permanent teeth eruption and growth complete.
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