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Incontinentia pigmenti: seven cases with dental manifestations
1Department of Orthodontics, John Radcliffe Hospital, Oxford, UK.
Insights
Incontinentia Pigmenti, a rare genetic skin disorder, often presents with significant dental abnormalities in female infants. This case series highlights common dental issues such as missing teeth and delayed eruption in affected children.
Area of Science:
- Dermatology
- Genetics
- Paediatric Dentistry
Background:
- Incontinentia Pigmenti (IP), also known as Bloch-Sulzberger syndrome, is a rare genodermatosis affecting primarily female infants.
- IP is characterized by distinct cutaneous stages and is often associated with systemic anomalies, including dental, ocular, and neurological complications.
Observation:
- A case series of seven female patients with IP presenting to a paediatric dentistry department over 16 years was reviewed.
- The study focused on the dental manifestations observed in these patients.
Findings:
- Common dental findings in the IP patients included hypodontia (missing teeth), microdontia (small teeth), and delayed tooth eruption.
- Two patients exhibited palatally impacted maxillary canines, indicating specific orthodontic concerns.
Implications:
- Early recognition of dental anomalies in IP is crucial for timely intervention and management.
- This case series underscores the importance of a multidisciplinary approach involving dentists, dermatologists, and other specialists for comprehensive patient care.
Abstract:
Incontinentia Pigmenti (Bloch-Sulzberger syndrome) is an uncommon genodermatosis that usually affects female infants. The condition is characterized by four cutaneous stages and is frequently associated with dental, ocular, central nervous system and structural anomalies. A large case series of seven patients, all female, who presented to the Department of Paediatric Dentistry at the Eastman Dental Hospital over the last 16 years is reported. The dental features of these cases were typical and included missing teeth, microdontia and delayed eruption. In two of the seven cases, both maxillary canines were palatally impacted.