Dental treatment considerations for a pediatric patient with incontinentia pigmenti (Bloch-Sulzberger syndrome)

ABSTRACT

Incontinentia pigmenti (IP) is a uncommon gene disorder, heritage with X-linked dominant mode. IP patients have a characteristic dentition varying from marked hypodontia to delayed eruption and conical crowns on both dentitions. A 5½-year-old girl, whose mother and younger sister were also diagnosed with IP, has the whirling-like pigmented skin lesion over her trunk and four extremities. Four primary teeth and multiple permanent tooth germs were found to be congenital missing. Dental considerations of further treatment were discussed with her parents including the preservation of primary molars, possible interim prosthesis in mixed or permanent dentition, full mouth rehabilitation with orthodontic and prosthodontic combined treatment, and implant therapy in adulthood. Early and longitudinal involvement of pediatric dentist to deal with the dental complications of IP can not only solve the esthetic problem and oral function but also maintain the oral health of children with IP to adulthood.

Key words:

Congenital missing - dermal pigmented lesion - hypodontia - incontinentia pigmenti

• REFERENCES

• 1 Smahi A, Courtois G, Vabres P, Yamaoka S, Heuertz S, Munnich A. et al. Genomic rearrangement in NEMO impairs NF-kappaB activation and is a cause of incontinentia pigmenti. The International incontinentia pigmenti (IP) Consortium. Nature 2000; 405: 466-72
• 2 Ehrenreich M, Tarlow MM, Godlewska-Janusz E, Schwartz RA. Incontinentia pigmenti (Bloch-Sulzberger syndrome): A systemic disorder. Cutis 2007; 79: 355-62
• 3 Landy SJ, Donnai D. Incontinentia pigmenti (Bloch-Sulzberger syndrome). J Med Genet 1993; 30: 53-9
• 4 Minic S, Trpinac D, Gabriel H, Gencik M, Obradovic M. Dental and oral anomalies in incontinentia pigmenti: A systematic review. Clin Oral Investig 2013; 17: 1-8
• 5 Kim BJ, Shin HS, Won CH, Lee JH, Kim KH, Kim MN. et al. Incontinentia pigmenti: Clinical observation of 40 Korean cases. J Korean Med Sci 2006; 21: 474-7
• 6 Okita M, Nakanishi G, Fujimoto N, Shiomi M, Yamada T, Wataya-Kaneda M. et al. NEMO gene rearrangement (exon 4-10 deletion) and genotype-phenotype relation in Japanese patient with incontinentia pigmenti and review of published work in Japanese patients. J Dermatol 2013; 40: 272-6
• 7 Hsiao PF, Lin SP, Chiang SS, Wu YH, Chen HC, Lin YC. NEMO gene mutations in Chinese patients with incontinentia pigmenti. J Formos Med Assoc 2010; 109: 192-200
• 8 Hadj-Rabia S, Froidevaux D, Bodak N, Hamel-Teillac D, Smahi A, Touil Y. et al. Clinical study of 40 cases of incontinentia pigmenti. Arch Dermatol 2003; 139: 1163-70
• 9 Al-Abdallah M, AlHadidi A, Hammad M, Al-Ahmad H, Saleh R. Prevalence and distribution of dental anomalies: A comparison between maxillary and mandibular tooth agenesis. Am J Orthod Dentofacial Orthop 2015; 148: 793-8
• 10 Bjerklin K, Al-Najjar M, Kårestedt H, Andrén A. Agenesis of mandibular second premolars with retained primary molars: A longitudinal radiographic study of 99 subjects from 12 years of age to adulthood. Eur J Orthod 2008; 30: 254-61
• 11 Park JH, Tai K, Yuasa K, Hayashi D. Multiple congenitally missing teeth treated with autotransplantation and orthodontics. Am J Orthod Dentofacial Orthop 2012; 141: 641-51