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Home Allgrove syndrome: a report of a unique case characterised by peculiar dental...

Allgrove syndrome: a report of a unique case characterised by peculiar dental findings resembling those of ectodermal dysplasia

Authors:

  • G. Tadini
    Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico di Milano, Department of Pathophysiology and Transplantation, University of Milan, Italy. Pediatric Dermatology Unit - Pediatric Clinic 1
  • F. Besagni
    Section of Dermatology, Department of Clinical and Experimental Medicine, Parma University, Parma, Italy
  • M. Callea
    Institute for Maternal and Child Health, IRCCS
  • M. Brena
  • L. C. Rossi
  • F. Angiero
  • R. Crippa

ABSTRACT


Aim

Triple A or Allgrove Syndrome (OMIM231550) is a rare, autosomal recessive genetic disorder in which patients
typically suffer from chronic adrenal insufficiency due to resistance to ACTH (Addison's disease), esophageal achalasia, and defective
tear formation (alacrima). The syndrome is caused by mutations in the AAAS gene on chromosome 12q13 encoding a 546 aminoacid
protein named alacrimia-achalasia-adrenal insufficiency neurologic disorder (ALADIN), a constituent of eukaryotic nuclear pore
complexes. CASE REPORT: We describe a case of Allgrove Syndrome presenting with anhidrosis and peculiar dental features
resembling those of Ectodermal Dysplasia (ED).

Conclusion

Given the clinical findings in this case we suggest the hypothesis that
the pathogenetic mechanism in Allgrove syndrome is related to the ED.

PLUMX METRICS

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Publication date:

Dec /2015

Keywords:

aaas gene, allgrove syndrome, conical shaped teeth, ectodermal dysplasia, enamel hypoplasia, hypodontia

Issue:

Vol.16 – n.4/2015

Page:

324 – 326

Publisher:

Ariesdue

Cite:


Harvard: G. Tadini, F. Besagni, M. Callea, M. Brena, L. C. Rossi, F. Angiero, R. Crippa (2015) "Allgrove syndrome: a report of a unique case characterised by peculiar dental findings resembling those of ectodermal dysplasia", European Journal of Paediatric Dentistry, 16(4), pp324-326. doi: https://www.ejpd.eu/wp-content/uploads/pdf/EJPD_2015_4_13.pdf
Vancouver: G. Tadini, F. Besagni, M. Callea, M. Brena, L. C. Rossi, F. Angiero, R. Crippa. Allgrove syndrome: a report of a unique case characterised by peculiar dental findings resembling those of ectodermal dysplasia. European Journal of Paediatric Dentistry [Internet]. 2015Dec.1 [cited 2023Feb.05];16(4):324-326. Available from: https://www.ejpd.eu/abstract-pubmed/allgrove-syndrome-a-report-of-a-unique-case-characterised-by-peculiar-dental-findings-resembling-those-of-ectodermal-dysplasia/
MLA: G. Tadini, F. Besagni, M. Callea, M. Brena, L. C. Rossi, F. Angiero, R. Crippa Allgrove syndrome: a report of a unique case characterised by peculiar dental findings resembling those of ectodermal dysplasia. European Journal of Paediatric Dentistry. 2015;16(4):324-326

Copyright (c) 2021 Ariesdue

Creative Commons License

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

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    European Journal of Paediatric Dentistry © | ISSN (Online): 2035-648X
    Registrazione del Tribunale di Milano n. 285 del 14.04.1998 | ROC 1946 - 26.09.2001
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