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James J, Thekkeveetil AK, Vadakkepurayil K. Oral Manifestations of Job's Syndrome in a Paediatric Dental Patient - A Case Report. J Clin Diagn Res. 2016;10(11):ZD04-ZD05doi: 10.7860/JCDR/2016/21340.8764.
James, J., Thekkeveetil, A. K., & Vadakkepurayil, K. (2016). Oral Manifestations of Job's Syndrome in a Paediatric Dental Patient - A Case Report. Journal of clinical and diagnostic research : JCDR, 10(11), ZD04-ZD05. https://doi.org/10.7860/JCDR/2016/21340.8764
James, Jeswin, et al. "Oral Manifestations of Job's Syndrome in a Paediatric Dental Patient - A Case Report." Journal of clinical and diagnostic research : JCDR vol. 10,11 (2016): ZD04-ZD05. doi: https://doi.org/10.7860/JCDR/2016/21340.8764
James J, Thekkeveetil AK, Vadakkepurayil K. Oral Manifestations of Job's Syndrome in a Paediatric Dental Patient - A Case Report. J Clin Diagn Res. 2016 Nov;10(11):ZD04-ZD05. doi: 10.7860/JCDR/2016/21340.8764. Epub 2016 Nov 01. PMID: 28050511; PMCID: PMC5198464.
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J Clin Diagn Res. 2016 Nov;10(11):ZD04-ZD05. doi: 10.7860/JCDR/2016/21340.8764. Epub 2016 Nov 01.

Oral Manifestations of Job's Syndrome in a Paediatric Dental Patient - A Case Report.

Journal of clinical and diagnostic research : JCDR

Jeswin James, Anupam Kumar Thekkeveetil, Kannan Vadakkepurayil

Affiliations

  1. Junior Resident, Department of Pedodontics, Government Dental College , Calicut, Kerala, India .
  2. Associate Professor, Department of Pedodontics, Government Dental College , Calicut, Kerala, India .
  3. Professor and Head, Department of Pedodontics, Government Dental College , Calicut, Kerala, India .

PMID: 28050511 PMCID: PMC5198464 DOI: 10.7860/JCDR/2016/21340.8764

Abstract

Job's syndrome or Hyperimmunoglobulin E Syndrome (HIES) is a rare primary immunodeficiency with marked increase in serum immunoglobulin E (IgE) levels and eczematous dermatitis. Individuals with HIES share characteristic facial features, with many oral manifestations like retained deciduous teeth, fissured tongue, missing permanent tooth buds, recurrent oral candidiasis, oral mucosal and gingival lesions. Two forms of disease have been identified based on the defective gene as autosomal dominant form and autosomal recessive form. The therapeutic strategies are usually directed towards prevention and management of recurrent infections which are refractory to the conventional strategies. About 200 cases of HIES have been reported so far, in the present case report authors present the oral manifestations of a paediatric dental patient affected with HIES.

Keywords: Hyperimmunoglobulin E syndrome; Immunoglobulin E; Recurrent infections

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