Tuyển tập báo cáo các nghiên cứu khoa học quốc tế ngành y học dành cho các bạn tham khảo đề tài: Incontinentia pigmenti presenting as hypodontia in a 3-year-old girl: a case report | Journal of Medical Case Reports BioMed Central Open Access Case report Incontinentia pigmenti presenting as hypodontia in a 3-year-old girl a case report Dárcio Kitakawa Patricia Campos Fontes Fernando Augusto Cintra Magalhães Janete Dias Almeida and Luiz Antonio Guimarães Cabral Address Department of Bioscience and Oral Diagnosis São José dos Campos Dental School São Paulo State University-UNESP São José dos Campos-SP Brazil Email Dárcio Kitakawa - dkitakawa@ Patrícia Campos Fontes - patty_cf@ Fernando Augusto Cintra Magalhães - magalhaesfac@ Janete Dias Almeida - janete@ Luiz Antonio Guimarães Cabral - cabral@ Corresponding author Published 10 November 2009 Received 30 April 2008 . z Accepted 10 November 2009 Journal of Medical Case Reports 2009 3 116 doi 1752-1947-3-116 This article is available from http content 3 1 1 16 2009 Kitakawa et al licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License http licenses by which permits unrestricted use distribution and reproduction in any medium provided the original work is properly cited. Abstract__ 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. However oral involvement characterized by hypodontia of deciduous and permanent teeth is important for the diagnosis and treatment of the patient. Case presentation We report the case of a 3-year-old girl with ophthalmologic and neurologic disturbances cutaneous .