Oral observations of hyper immunoglobulin-e syndrome: A case report

Abstract

The hyperimmunoglobulin E syndrome is a rare primary immunodeficiency, characterized by a triad of clinical signs including elevated serum IgE levels, recurrent staphylococcal skin abscesses and pneumonia. It can be autosomal dominant or recessive and is characterized by immunological and non-immunological abnormalities.

Individuals with HIES share characteristic facial features, with many oral manifestations like retained deciduous teeth, missing permanent tooth buds, high arched palate, oral and gingival lesions, etc.

This report aimed to describe a rare clinical case of a 13-year-old girl, that was referred to our paediatric dentistry department at the Mohammed V University Hospital in Rabat-Morocco, diagnosed with autosomal dominant hyperimmunoglobulin E syndrome with numerous oral manifestations.