Oral Manifestations of Job's Syndrome in a Paediatric Dental Patient - A Case Report.
J Clin Diagn Res. 2016 Nov;10(11):ZD04-ZD05
Authors: James J, Thekkeveetil AK, Vadakkepurayil K
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.
PMID: 28050511 [PubMed]