incontinentia pigmentosa (IP), also known as incontinence pigmentosa, is a rare X-chromosome-linked dominant genetic disease caused by mutations in the IKBKG gene located at Xq28, with an incidence of about 1/50,000, mainly in women[1,2]。 The disease mainly leads to abnormal ectodermal development with characteristic skin changes, which can be accompanied by deformities and abnormalities of the hair, nails, teeth, eyes and central nervous system[3]。 Among the patients with IP, 54.38% had abnormal oral manifestations, 31.22% had congenital missing teeth and 36.42% had abnormal teeth[2]。 A 7-year-old IP patient with congenital missing teeth was admitted to the Department of Children's Stomatology, School of Stomatology, China Medical University. He was treated with comprehensive oral treatment and genetic testing at the request of his parents. The report is as follows.
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