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.