INCONTINENTA PIGMENTI IN A 10 YEAR-OLD GIRL

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.