Oral Manifestations of Job’s Syndrome in a Paediatric Dental Patient – A Case Report

Abstract

A 12-year-old boy was referred to the Department of Pedodontics, with the chief complaint of multiple decayed teeth. Detailed physical examination by the Paediatrician revealed numerous skin abscesses and pruritic dermatitis on scalp, face, limbs and trunk. The nails were showing definitive koilonychia with no evidence of icterus, cyanosis, lymphadenopathy and pedal oedema.Medical history revealed episodes of recurrent abscesses in different parts of the body, associated with recurrent fever, cough and episodes of haemoptysis with the pus culture showing positivity for Staphylococcus aureus. Computed Tomography (CT) scan report of thorax showed thick walled bronchiectatic cavities with air fluid level in outer segments of right upper lobe and post-basal segment of right lower lobe. Haematological examination revealed that the white blood cell count was 19240 cells/µL with 71% neutrophils, and an elevated eosinophils level at 12%. Haemoglobin level was at 8.9 gram% with haematocrit at 28.9%. The serum Immunoglobulin E (IgE) level showed elevation to 16,025 IU/ml with the other immunoglobulins at normal range except for the reduction in IgM level.Facial and intra-oral examination revealed the characteristic coarse facial form, prominent forehead, rough facial skin, deep set wide spaced eyes, broad nasal bridge, wide fleshy nasal tip, Class III skeletal pattern, mild facial asymmetry with an old man appearance [Table/ Fig-1]. Mild cervical lymphadenopathy, angular cheilitis, depapillation and fissuring of the tongue [Table/ Fig-2], high arched palate, multiple keratotic/erosive areas on tongue and buccal mucosa, multiple non-specific ulcers on gingiva and poor oral hygiene were also noted. Dental examination revealed multiple carious lesions and root stumps of teeth # 52, 62, 71, 72, 81 and 82 with poor oral hygiene. Orthopantomogram (OPG) revealed absence of permanent tooth buds of teeth # 12, 22, 31, 32, 41, 42 and a supernumerary tooth in relation to tooth # 34 [Table/ Fig-3]. Extraction of root stumps and necessary restorations were done to eliminate all infectious foci. The patient was referred back to the Department of Paediatrics for further evaluation.

Orodental health status of patients with inborn errors of immunity

“…For example, in addition to skin eczema and recurrent infections, patients with autosomal dominant hyper-IgE syndrome (AD-HIES) have a distinctive face, class III occlusion, and prolonged primary tooth retention. 10 Furthermore, IEI may be associated with enamel hypoplasia, missing teeth, and malocclusion. 7 To our knowledge, there are no public oral health programs for patients with IEI.…”

“…In addition to infection problems, patients may also experience developmental abnormalities 9 that are a characteristic of syndromic disease. For example, in addition to skin eczema and recurrent infections, patients with autosomal dominant hyper‐IgE syndrome (AD‐HIES) have a distinctive face, class III occlusion, and prolonged primary tooth retention 10 . Furthermore, IEI may be associated with enamel hypoplasia, missing teeth, and malocclusion 7 .…”