Oral findings in a patient with incontinentia pigmenti (Bloch-Sulzberger syndrome)

Incontinentia pigmenti, also known as Bloch-Sulzberger syndrome, is an uncommon X-linked dominant disorder that affects mostly females and is usually lethal in males in utero. The disease is characterized by abnormalities of the skin, eyes, central nervous system and dentition. Dental abnormalities are the most common non-cutaneous manifestation of the disease, occurring in more than 80% of all patients. This report describes a patient with incontinentia pigmenti who exhibited late dentition, multiple missing and malpositioned conical teeth in both arches. Early and repeated dental intervention may be required since significant absence and malformation of teeth can affect proper facial development, mastication, speech development and result in nutritional deficiency and growth retardation in very severe cases. Referral for radiological evaluation and dental intervention by the age of 2 years is appropriate to establish adequate dental treatment for the affected children. There should be interdisciplinary coordination, leading to better treatment and therefore an improved quality of life for these patients.