Case report: Incontinence Pigmenti or Bloch-Sulzberger Syndrome.

Abstract

Incontinentia pigmenti (PI), also known as Bloch-Sulzberger Syndrome or Bloch-Siemens Syndrome, is a rare genodermatosis characterized by alterations in the ecto and mesodermal tissues of different organs and systems, with cutaneous, ocular, dental and systemic manifestations. central nervous. PI has an autosomal dominant inheritance linked to the X chromosome and affects females, in more than 95% of cases. Case report of a female neonate diagnosed at an early stage of the disease from compatible cutaneous manifestations and anatomopathological examination, ensuring adequate long-term multidisciplinary management.