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Incontinentia pigmenti: exploring the oral manifestations
Yashika Jain1, Vidya Ajila2, G Subhas Babu1
1Department of Oral Medicine and Radiology, NITTE (Deemed to be University), AB Shetty Memorial Institute of Dental Sciences (ABSMIDS), Mangalore, Karnataka, India.
BMJ Case Reports
|May 29, 2026
Summary
Incontinentia pigmenti (IP) is a rare X-linked genetic disorder affecting neuroectodermal tissues. This case highlights typical dental anomalies in an adolescent girl diagnosed with IP, including missing teeth and delayed eruption.
Area of Science:
- Genetics
- Dermatology
- Dentistry
Background:
- Incontinentia pigmenti (IP) is a rare, X-linked genetic disorder.
- It primarily affects ectodermal structures like skin, teeth, eyes, hair, nails, and the central nervous system.
- Dermatological and dental manifestations are common, with skin issues often appearing first.
Purpose of the Study:
- To discuss a case of Incontinentia pigmenti (IP) in an adolescent female.
- To highlight the characteristic dental findings associated with IP.
Main Methods:
- Case report of an adolescent girl diagnosed with IP.
- Clinical examination focusing on dermatological and dental features.
Main Results:
- The patient presented with typical dental manifestations of IP.
- Observed dental anomalies included missing permanent teeth, retained deciduous teeth, microdontia, and delayed eruption.
Conclusions:
- Incontinentia pigmenti (IP) presents with significant and often characteristic dental abnormalities.
- Early recognition of dental features can aid in the diagnosis of IP.
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