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[Hyper IgE syndrome: three case reports]. 2014

Maria Teresa Tagle C, and Alvaro Melys G, and Angela Castillo M, and Ximena Norambuena R, and Arnoldo Quezada L

BACKGROUND Autosomal dominant Hyper IgE syndrome (HIES-AD) is a primary immunodeficiency associated with connective tissue, skeletal, vascular and brain disorders. The pathogenesis of immune deficiency lies in an alteration of Th17 cells which explains the special susceptibility of these patients to S. aureus and Candida infections. OBJECTIVE To describe three children diagnosed with hyper IgE syndrome and conduct a study on the subject, with special focus on the dominant form of the disease. METHODS 3 children with HIES-AD (2 males and one female) with eczema since birth, skin, ear, lung, and lymph node infections, and serum IgE levels over 2,000 IU/ml and eosinophilia values, treated with antibiotics and topically, and 7 year follow-up. CONCLUSIONS It is a rare condition that requires a high index of suspicion and early management of infections. One of its main diagnoses is atopic syndrome with recurrent infections but both conditions differ in context, response and resolution against infections and lack of other phenotypic characteristics.

UI MeSH Term Description Entries
D007073 Immunoglobulin E An immunoglobulin associated with MAST CELLS. Overexpression has been associated with allergic hypersensitivity (HYPERSENSITIVITY, IMMEDIATE). IgE
D007223 Infant A child between 1 and 23 months of age. Infants
D007589 Job Syndrome 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. HIES, Autosomal Recessive,Hyper-IgE Syndrome, Autosomal Recessive,Hyper-Immunoglobulin E Syndrome, Autosomal Recessive,Hyperimmunoglobulin E-Recurrent Infection Syndrome,Job's Syndrome,Buckley Syndrome,HIE Syndrome,HIES, Autosomal Dominant,Hyper-IgE Recurrent Infection Syndrome, Autosomal Recessive,Hyper-IgE Syndrome,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
D008297 Male Males
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D004485 Eczema A dermatitis characterized by a spongiotic tissue reaction pattern occurring as a reaction to many endogenous and exogenous agents. Dermatitis, Eczematous,Eczematous Dermatitis
D005260 Female Females
D005500 Follow-Up Studies Studies in which individuals or populations are followed to assess the outcome of exposures, procedures, or effects of a characteristic, e.g., occurrence of disease. Followup Studies,Follow Up Studies,Follow-Up Study,Followup Study,Studies, Follow-Up,Studies, Followup,Study, Follow-Up,Study, Followup
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

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