PMID- 28050511 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20201001 IS - 2249-782X (Print) IS - 0973-709X (Electronic) IS - 0973-709X (Linking) VI - 10 IP - 11 DP - 2016 Nov TI - Oral Manifestations of Job's Syndrome in a Paediatric Dental Patient - A Case Report. PG - ZD04-ZD05 LID - 10.7860/JCDR/2016/21340.8764 [doi] AB - 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. FAU - James, Jeswin AU - James J AD - Junior Resident, Department of Pedodontics, Government Dental College , Calicut, Kerala, India . FAU - Thekkeveetil, Anupam Kumar AU - Thekkeveetil AK AD - Associate Professor, Department of Pedodontics, Government Dental College , Calicut, Kerala, India . FAU - Vadakkepurayil, Kannan AU - Vadakkepurayil K AD - Professor and Head, Department of Pedodontics, Government Dental College , Calicut, Kerala, India . LA - eng PT - Case Reports DEP - 20161101 PL - India TA - J Clin Diagn Res JT - Journal of clinical and diagnostic research : JCDR JID - 101488993 PMC - PMC5198464 OTO - NOTNLM OT - Hyperimmunoglobulin E syndrome OT - Immunoglobulin E OT - Recurrent infections EDAT- 2017/01/05 06:00 MHDA- 2017/01/05 06:01 PMCR- 2017/01/01 CRDT- 2017/01/05 06:00 PHST- 2016/05/12 00:00 [received] PHST- 2016/07/11 00:00 [accepted] PHST- 2017/01/05 06:00 [entrez] PHST- 2017/01/05 06:00 [pubmed] PHST- 2017/01/05 06:01 [medline] PHST- 2017/01/01 00:00 [pmc-release] AID - 10.7860/JCDR/2016/21340.8764 [doi] PST - ppublish SO - J Clin Diagn Res. 2016 Nov;10(11):ZD04-ZD05. doi: 10.7860/JCDR/2016/21340.8764. Epub 2016 Nov 1.