Clinical features of incontinentia pigmenti with emphasis on oral and dental abnormalities

One of interesting aspects in dermatology is the fact that skin may reflect the presence of anomalies in other organs and tissues. One such example is incontinentia pigmenti (IP), a rare, complex, X-linked genodermatosis. Clinical manifestations of IP according to evolution and prognosis can be considered as skin, as well as dental, eye, and central nervous system, changes. We have investigated a total of nine families with 25 subjects, 23 females and 2 males. In 12 female and 2 male subjects, all of them with clinical characteristics of IP, we observed the following abnormalities: teeth-shape anomalies (coni- or peg-like teeth), the presence of numerous cariotic teeth, early dental loss, delayed eruption, partial anodontia, and gothic palate. To our knowledge, this is the first time that the presence of gothic palate in patients with IP has been documented. As we found out, in two female subjects and one male subject, in which nonrandomed X inactivation did not occur, gothic palate could be supposed as characteristic of IP.