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Abstract
A 13-year-old female patient went to the pediatric dentistry service of the Oral Health Institute of the Central Air Force Hospital of Peru in September of 2018.
She sought care to have her temporary partial prosthesis renewed. The extraoral clinical examination showed scar lesions on the skin of the nasal region; the intraoral evaluation showed the loss of multiple teeth, wide and thin edentulous ridges, alterations in dental morphology, as well as the inconsistency of the dental eruption with the patient’s age. The radiographic examination showed alteration and delay in dental eruption, impaction of a tooth in the jaw, lack of root development and persistence of temporary teeth. The definitive medical diagnosis was BlochSulzberger Syndrome, also referred to as “pigmentary incontinence”. This syndrome is characterized by presenting a variety of oral manifestations such as dental agenesis, alteration in dental morphology, retained and/or impacted teeth as well as alteration and delay in dental eruption. At a systemic level, this syndrome has lesions that affect the skin, hair, vision, central nervous system, among others.
References
Bloch-Sulzberger syndrome. Hautarzt. 2010;
61 (10): 831-3.
2. Morrell DS, Burkhart CN, Siegel D. Enfermedades hereditarias seleccionadas. En: Eichenfield L, Frieden I, Esterly N. (eds.). Dermatologia Neonatal. 2da ed. Barcelona, España:
Elsevier; 2009. p. 485.
3. Pipa VA, González GM, López-Arranz ME,
Fernández TJ. Incontinencia Pigmentaria.
Consideraciones odontoestomatológicas: Profilaxis y terapéutica. Av Odontoestomatol. 2005;
21 (4): 211-5.
4. García DJ, De Unamuno P, Fernández LE,
Salazar VJ, Armijo M. Incontinentia pigmenti: XXY male with family history. Clin Genet.
1990; 38 (2): 128-38.
5. Ormerod AD, White MI, McKay E, Johnston AW. Incontinentia pigmenti in a boy with
Klinefelter´s syndrome. J Med Genet. 1987; 24
(7): 39-41.
6. Landy SJ, Donnai D. Incontinentia pigmenti (Bloch-Sulzberger syndrome). J Med Genet.1993; 30 (1): 53-9.
7. Geiges ML. Traces of Marion B. Sulzberger in
the Museum of Wax Moulages in Zurich and
their importance for the history of dermatology.
J Am Acad Dermatol.2009; 60 (6): 980-4.
8. The International Incontinentia Pigmenti
(IP) Consortium. Genomic rearrangement in
NEMO impairs NF-(kappa)B activation and
is a cause of incontinentia pigmenti. Nature.
2000;405 (6785): 466-72.
9. Kenwrick S, Woffendin H, Jakins T, Shuttleworth SG, Mayer E, Greenhalgh L, Whittaker
J, Rugolotto S, Bardaro T, Esposito T, D’Urso
M, Soli F, Turco A, Smahi A, Hamel-Teillac D,
Lyonnet S, Bonnefont JP, Munnich A, Aradhya
S, Kashork CD, Shaffer LG, Nelson DL, Levy
M, Lewis RA; International IP Consortium.
Survival of male patients with incontinentia
pigmenti carrying a lethal mutation can be
explained by somatic mosaicism or Klinefelter
syndrome. Am J Hum Genet. 2001; 69 (6):
1210-7.
10. Chang JT, Chiu PC, Chen YY, Wang HP,
Hsieh KS. Multiple clinical manifestations and
64
Yuri Castro-Rodríguez, Jose Cósar-Quiroz, Elizabhet Julcamoro-Grados
diagnostic challenges of incontinentia pigmenti
- 12 years’ experience in 1 medical center. J
Chin Med Assoc. 2008; 71(9): 455-60.
11. Carney RG. Incontinentia pigmenti: A world
statistical analysis. Arch Dermatol.1976; 112
(4): 535-42.
12. Gómez VR, Juárez M, Plascencia GA, Reyes
MG, Saénz CC, Káram M et al. Incontinentia
pigmenti. Artículo de revisión. Dermat Cosmet
Méd Quir. 2010;8 (2): 104-9.
13. Rouse JS. Incontinentia pigmenti: overcoming
cosmetic challenges. Compend Contin Educ
Dent.1999; 20 (11): 1034-6.
14. Rouse JS. Incontinentia pigmenti: overcoming
cosmetic challenges. Compend Contin Educ
Dent. 1999; 20 (11): 1040-1.
15. Berlin AL, Paler AS, Chan LS. Incontinentia
pigmenti: A review and update on the molecular
basis of pathophysiology. J Am Acad Dermatol.
2002; 47(2): 169-87.
16. Kasmann KB, Jurin BB, Ruprech KW.
Incontinentia pigmenti (Bloch-Schulzberger
syndrome): case report and differential
diagnosis to related dermato-ocular syndroms.
Ophthalmologica. 1999; 213 (1): 63-9.
17. Romero A, Tufiño M, Villacís A, Salazar M.
Incontinencia pigmentaria o síndrome de
Bloch-Sulzberger. Dermatol Rev Mex. 2014;
58 (6): 539-43.
18. Atamari AN, Solórzano GS. Incontinentia
pigmenti (Síndrome de BlochSulzberger) en un
paciente varón. Reporte de un caso. Rev Med
Hered. 2015;26 (4): 238-41.
19. Maino BG, Pagin P, Mura P. Spider Screw ®:
Anclaje absoluto de carga inmediata. Rev Esp
Ortod. 2003;33 (1): 21-30.
20. Puigdollers A. Anclaje estable sobre implantes
palatinos. Rev Esp Ortod. 2003; 33 (2): 121-31.
21. De Rábago JA, Sanz M. Manejo
multidisciplinario de las agenesias dentarias.
Periodoncia. 2003;13 (5): 399-412.