A Case of Hyper Immunoglobulin-E Syndrome Associated with Scrotal Tongue and Intraoral Hyperpigmentation

[3] While multisystem involvement is the rule in sporadic and autosomal dominant (AD-HIES) types, autosomal recessive (AR-HIES) cases have severe viral infections and potentially lethal neurologic complications but lack skeletal and dental involvement. [4]{Figure 4} Based on the above, diagnosis of AD-HIES was made and the patient gradually improved with treatment consisting of antibiotics, antifungals, emollients and oral vitamin C. After the initial description by Davis et al., of two patients with red hair, severe chronic dermatitis, cold abscesses and recurrent pneumonia, Buckley et al. in 1972 defined the classical triad of Job's syndrome comprising of extremely high IgE levels, eczema and recurrent skin and lung infections; hence the eponym of Buckley's syndrome for this entity.