Job’s syndrome with an atypical presentation

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.