Primary immunodeficiency syndrome characterized by recurrent infections and hyperimmunoglobulinemia E. Most cases are sporadic. Of the rare familial forms, the dominantly inherited subtype has additional connective tissue, dental and skeletal involvement that the recessive type does not share.
Also Known As:
Hyper-IgE Syndrome; Buckley Syndrome; HIE Syndrome; HIES, Autosomal Dominant; Hyper-IgE Recurrent Infection Syndrome, Autosomal Recessive; Hyper-IgE Syndrome, Autosomal Dominant; Hyper-Immunoglobulin E Syndrome, Autosomal Dominant; Hyperimmunoglobulin E, Recurrent Infection Syndrome; Hyperimmunoglobulinemia E Syndrome; Job-Buckley Syndrome; Autosomal Dominant HIES; Autosomal Dominant HIESs; Autosomal Recessive HIES; Autosomal Recessive HIESs; Buckley Syndromes; HIE Syndromes; HIESs, Autosomal Dominant; HIESs, Autosomal Recessive; Hyper IgE Recurrent Infection Syndrome, Autosomal Recessive; Hyper IgE Syndrome; Hyper IgE Syndrome, Autosomal Dominant; Hyper IgE Syndrome, Autosomal Recessive; Hyper Immunoglobulin E Syndrome, Autosomal Dominant; Hyper Immunoglobulin E Syndrome, Autosomal Recessive; Hyper-IgE Syndromes; Hyperimmunoglobulin E Recurrent Infection Syndrome; Hyperimmunoglobulinemia E Syndromes; Job Buckley Syndrome; Job Syndromes; Job-Buckley Syndromes; Jobs Syndrome; Syndrome, Job; Syndromes, Job; HIES, Autosomal Recessive; Hyper-IgE Syndrome, Autosomal Recessive; Hyper-Immunoglobulin E Syndrome, Autosomal Recessive; Hyperimmunoglobulin E-Recurrent Infection Syndrome; Job's Syndrome