Introduction
Autosomal dominant hyper-IgE syndrome (AD-HIES), also known as Buckley or Job’s syndrome, is a rare multisystem disorder with both immunologic and nonimmunological characteristics. 1 This disorder is largely associated with heterozygous dominant-negative mutations in signal transducer and activator of transcription 3 (STAT3 ) gene and characterized by eczematoid rashes, staphylococcal skin abscesses, connective tissue defects, and extremely elevated IgE serum level. 2-5
Abnormalities in the dentition are often observed in patients with AD-HIES, such as retention of primary teeth together with ectopic eruption or non-eruption of permanent teeth. 6 In addition to dental abnormalities, some oral cavity defects have been reported for AD-HIES, including deep grooves on the tongue, buccal mucosa with multiple fissures, central ridges and fissures of the palate and a high arched palate. 7
In this report, we reported dental abnormalities observed in an AD-HIES patient suffering from dermatitis, asthma, eczema, anaphylaxis, and refractory sinusitis.