Introduction: Incontinentia pigmenti (IP) or Bloch–Sulzberger syndrome is a rare X-linked disorder that involves ectodermaltissues. Disease mainly affects the skin, eyes, hair, and central nervous system. Oral involvement is characterized by hypodontiaof deciduous and permanent teeth.Case presentation: We are reporting a very rare case of IP in an 11 year old boy having hyperpigmented lesions on head, neckand trunk. Intraoral examination revealed partially erupted tooth, multiple missing teeth and crowding of lower anterior teeth.Management and prognosis: The patient was later referred to department of pedodontia for full mouth rehabilitation.Conclusion: The information from this case report increase awareness among health care providers like general dentist, oralphysicians, pediatricians and dermatologists.
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