Incontinenta pigmenti (Bloch-Sulzberger disease) در یک دختر 10 ساله

  Abstract   Incontinenta pigmenti or Bloch-Sulzberger syndrome is a rare x-linked dominant disease that mainly affects the skin, eye, hair, central nervous system and teeth. The disease is predominant among women. Dermatologic manifestations are among the most important aspects for the diagnosis of the syndrome. Oral involvement characterized by hypodontia of deciduous and permanent teeth is important for diagnosis and treatment of the patient. We report the case of a 10-year-old girl with cutaneous manifestation and hypodontia. The ophthalmologic and neurologic disturbances were not significant and the chief complaint of the patient was missing teeth. The diagnosis was established according to the history, physical examination, and radiography of teeth.   SOURCE: URMIA MED J 2013: 24(1): 78 ISSN: 1027-3727