title: Job's syndrome with an atypical presentation.
abstract: Hyper Immunoglobulin E syndrome also called Job's or Buckley Syndrome is a rare primary immunodeficiency disease characterized by elevated serum IgE levels (> 2000 IU/ml), recurrent infections and eosinophilia. Other features include coarse facies and non-immunologic abnormalities of the dentition, bones, vasculature and connective tissues. We are reporting a case of a twenty four years old male with coarse facies who presented with severe pallor and upper gastrointestinal bleeding. Investigations revealed markedly elevated serum IgE levels (11,800 IU/ml), severe anaemia, esophageoduodenal erosions, Helicobacter pylori (H. pylori) gastritis and oro-esophageal candidiasis.
authors.affiliation: Department of Hepatogastroenterology, Aga Khan Hospital for Woman, Karimabad. draayt@yahoo.com
authors.forename: Abbas Ali
authors.initials: AA
authors.lastname: Tasneem
authors.affiliation: Department of Diagnostics and Immunology, Aga Khan Hospital for Woman, Karimabad.
authors.forename: Sabahat
authors.initials: S
authors.lastname: Sarfaraz
authors.affiliation: Department of Hepatogastroenterology, Aga Khan Hospital for Woman, Karimabad.
authors.forename: Syed Mujahid
authors.initials: SM
authors.lastname: Hassan
authors.affiliation: Department of Hepatogastroenterology, Aga Khan Hospital for Woman, Karimabad.
authors.forename: Nasir Hassan
authors.initials: NH
authors.lastname: Luck
authors.affiliation: Department of Diagnostics and Immunology, Aga Khan Hospital for Woman, Karimabad.
authors.forename: Sabiha
authors.initials: S
authors.lastname: Anis
authors.affiliation: Department of Hepatogastroenterology, Aga Khan Hospital for Woman, Karimabad.
authors.forename: Zaigham
authors.initials: Z
authors.lastname: Abbas
mesh_terms: D019066:Facies; D006801:Humans; D007589:Job Syndrome / Q000150:complications* / Q000175:diagnosis* / Q000628:therapy; D008297:Male; D055815:Young Adult