Clinical manifestations in the oral cavity in patients with hyper-IgE syndrome.
Dorota Olczak-Kowalczyk, Anna Matosek, Edyta Heropolitańska-Pliszka, Ewa Bernatowska
(Centr Eur J Immunol 2013; 38 (1): 92-99)
DOI (digital object identifier): 10.5114/ceji.2013.34363
Introduction: Hyper-IgE syndrome is a rare an autosomal recessive or dominant manner due to gene mutations. This syndrome, from mutations in the STAT3 gene, is characterized by elevated levels of IgE > 2000 IU/ml, eczema, skin abscesses, recurrent respiratory infections, skeletal abnormalities, oral mucosal lesions, impaired eruption of permanent teeth and root resorption of deciduous teeth.
Aim of the study: Determine phenotypic characteristics of hyper-IgE syndrome in the oral cavity with regard to a modifying impact of environmental factors.
Results: Examination of the oral mucosa revealed white lichenoid lesions, atrophy of the lingual papillae, median schistoglossia, palatine fibrosis, erosions, ulceration and scarring, angular cheilitis. Candida albicans was identified, despite antimycotic treatment. Dental examination revealed caries, unerupted teeth, persistent deciduous teeth, and tooth wear. The phenotypic variability in the oral cavity might have been due to environmental factors.
Conclusions: Although the genetic causes of hyper-IgE have been identified, the pathogenesis of oral lesions in those patients remains to be clarified. The current knowledge allows associating the oral mucosal lesions, i.e., fungus infections, hyperkeratosis and fibrosis, with the STAT3 gene mutation.
It also helps to consider its role in odontogenic disorders, particularly in inhibiting eruption of permanent teeth and root resorption in deciduous dentition.
Aim of the study: Determine phenotypic characteristics of hyper-IgE syndrome in the oral cavity with regard to a modifying impact of environmental factors.
Results: Examination of the oral mucosa revealed white lichenoid lesions, atrophy of the lingual papillae, median schistoglossia, palatine fibrosis, erosions, ulceration and scarring, angular cheilitis. Candida albicans was identified, despite antimycotic treatment. Dental examination revealed caries, unerupted teeth, persistent deciduous teeth, and tooth wear. The phenotypic variability in the oral cavity might have been due to environmental factors.
Conclusions: Although the genetic causes of hyper-IgE have been identified, the pathogenesis of oral lesions in those patients remains to be clarified. The current knowledge allows associating the oral mucosal lesions, i.e., fungus infections, hyperkeratosis and fibrosis, with the STAT3 gene mutation.
It also helps to consider its role in odontogenic disorders, particularly in inhibiting eruption of permanent teeth and root resorption in deciduous dentition.
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