Severe atopic eczema and recurrent infections:diagnosis of the Job syndrome according to Guimbacher criteria

Introduction The syndrome of hyper-IgE syndrome or Job syndrome or Burckley syndrome, is a rare primary immunodeficiency characterized by defective phagocytic manifesting recurrent infections, mainly Staphylococcal associated with eczema severe atopic, musculoskeletal disorders, pathological fractures, scoliosis and levels high IgE (> 2.000UI / ml). Among the immunological characteristics, we can find defects in neutrophil chemotaxis, decreased production of interferon gamma, eosinophilia mutation and STAT 3.Most cases are sporadic, but there are cases with patterns of autosomal dominant or recessive inheritance. The diagnosis is clinical using the criteria of Grimbacher.The treatment is the same of the bacterial and fungal infections. Some consensus oriented prophylaxis of infections with antibiotics.