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

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