title: A Boy with Relentless Pruritus: Job's Syndrome.
abstract: BACKGROUND
  Job's syndrome (hyper IgE syndrome) is a very rare primary immunodeficiency disease that has an annual approximate incidence of less than 1/1,000,000. This manuscript aims to provide education regarding diagnosis and management strategies of this syndrome worldwide.
  
  
  METHODS
  A 6-year-old boy was seen at the clinic secondary to persistent pruritus interfering with sleep. At the age of 2 months, the patient developed diffuse eczematous and desquamating skin lesions. He was subsequently diagnosed with atopic dermatitis and managed conservatively. From 2 months to 7 years of age, intermittent exacerbations of dermatitis persisted despite an aggressive treatment regimen. The serum IgE level increased exponentially over a period of 7 years, with a peak value of 57,400 IU/ml. Molecular genetic testing revealed a dominant negative mutation within the SH2 domain of the Signal Transducer and Activator of Transcription (STAT3) gene. The patient was subsequently diagnosed with Job's syndrome. Management included proper skin care, prophylactic antibiotics, immunomodulating agents, and psychotherapy.
  
  
  CONCLUSIONS
  Job's syndrome can often go unrecognized and masquerade as atopic dermatitis. Therefore, genetic testing for this condition should be obtained in all patients with treatment-refractory AD. Additionally, psychotherapy can be a successful management strategy for the grating psychological impact that can be imposed on children with excessive pruritus.
authors.affiliation: Department of General Surgery, Sinai Hospital of Baltimore, Baltimore, MD, USA.
authors.forename: Kamran
authors.initials: K
authors.lastname: Khan
authors.affiliation: Department of General Surgery, Sinai Hospital of Baltimore, Baltimore, MD, USA.
authors.forename: Susan E
authors.initials: SE
authors.lastname: Wozniak
authors.affiliation: , Aureus University School of Medicine, Oranjestad, Aruba.
authors.forename: Anna Lucia
authors.initials: AL
authors.lastname: Giannone
authors.affiliation: Psychoanalysis, Private Practice, Lechería, Anzoategui, Venezuela.
authors.forename: Maria Elena
authors.initials: ME
authors.lastname: Abdulmassih
mesh_terms: D002648:Child; D003876:Dermatitis, Atopic / Q000175:diagnosis; D003937:Diagnosis, Differential; D006801:Humans; D007073:Immunoglobulin E / Q000097:blood; D007589:Job Syndrome / Q000175:diagnosis* / Q000235:genetics; D008297:Male; D009154:Mutation; D011537:Pruritus / Q000209:etiology*; D050796:STAT3 Transcription Factor / Q000235:genetics
chemical_list: D050796:STAT3 Transcription Factor; C494086:STAT3 protein, human; D007073:Immunoglobulin E