INTRODUCTION Incontinentia pigmenti or Bloch-Sulzberger syndrome is a rare X-linked dominant disease that mainly affects the skin, eyes, hair, central nervous system and teeth. The disease is predominant among women. Although dermatologic manifestations are among the most important aspects for the diagnosis of the syndrome, they are less damaging to the patient and do not require treatment. How...

The course and histologic skin signs are described at the stage of inflammation in 2 cases of incontinentia pigmenti (IP) or the Bloch-Sulzberger syndrome. In the authors' opinion, the pigmentary stage of skin lesion in IP does not replace the warty stage, but accompanies it or the inflammatory one as blebs disappear. In IP, the latter contain eosinophils; there is plasma and cell infiltration ...

Incontinentia pigmenti (IP) is a genodermatosis with an X-linked dominant mode of inheritance, characterized by ectodermal, mesodermal, neurological, ocular, and dental manifestations. The purpose of this case study was to report the oral and dental manifestations of an IP case in a Venezuelan pediatric patient. A 9 year-old Venezuelan girl was evaluated. She showed macular pigmented lesions in...