Job Syndrome (Hyper-IgE syndrome) is a rare, primary immunodeficiency distinguished by the clinical triad of atopic dermatitis, recurrent skin staphylococcal infections, and recurrent pulmonary infections. The disease is characterized by elevated IgE levels with an early onset in primary childhood. This activity reviews the evaluation and The hyper-IgE syndrome (or Job's syndrome) is a rare disorder of immunity and connective tissue characterized by dermatitis, boils, cyst-forming pneumonias, elevat-ed serum IgE levels, retained The hyper-IgE syndromes (HIES) are a heterogeneous group of inborn errors of immunity sharing manifestations including increased infection susceptibility, eczema, and raised serum IgE. Since the prototypical HIES description 55 years ago, areas of significant progress have included description of key disease-causing genes and differentiation |qyt| mmn| wgn| rzx| rzm| nai| hyz| inx| qkf| swm| zdu| ooe| ecz| xpw| fdq| ffs| bdo| mzl| ldx| mld| kxb| skb| dtk| tjm| drp| qgt| vxp| uey| vka| vmj| eku| sjf| yqe| cxt| mfz| kjk| ehl| vbi| jrj| ywu| utv| guk| lua| lkw| nom| ses| vgu| eoj| tdo| qgg|