Incontinentia pigmenti: case report and 5-year follow-up

Abstract

Introduction: Incontinentia Pigmenti (Bloch-Sulzberger Syndrome) is a disease of organ involvement with ectodermic and mesodermic origin, showing dominant transition based on the X-chromosome. It affects the skin, eyes, teeth, and central nervous system. Case Report: The patient diagnosed with Incontinentia Pigmenti was referred from the Department of Dermatology, Faculty of Medicine, Mustafa Kemal University to the Department of Periodontology, Faculty of Dentistry, Mustafa Kemal University to consult whether there was anomaly in tooth germs and crests in the mouth. The patient was followed in our clinic for 5 years (examinations at 42 days, 6 months and 5 years). In the extraoral examinations, second-grade hyperkeratotic erythematous papules and plaques on the arms and legs, and linearly-arranged hyperpigmented and hypopigmented macular lesions at various grades were seen. In the intraoral examinations, no anomalies were observed in the crests and the palate during the toothless period. However, during the dentition period, wedge-shaped teeth and oligodontia were observed. In the 5-year follow-up period, no deficiency was observed in the patient's body and mental development compared with the peers. Conclusion: Early diagnosis of Incontinentia Pigmenti can be made through observation of the dental anomalies. There is no specific treatment of this disease. As it affects multiple systems of the human body, it requires monitoring and treatment with interdisciplinary approaches.

Keywords

• Bloch-Sulzberger syndrome
• Incontinentia Pigmenti
• tooth abnormalities