Atypical celiac disease with IgA deficiency presenting as Plummer–Vinson syndrome: a case report

Abstract

A 40-year-old man presented with insidious onset dysphagia for both solids and liquids for 4 years with recurrent oral ulcerations. On examination he was anemic, and barium swallow demonstrated a web in the postcricoid region. As part of the workup for unexplained iron deficiency anemia, a duodenal biopsy was taken that revealed moderate flattening of villi with increased intraepithelial lymphocytes consistent with the diagnosis of celiac disease. However, the serological tests for celiac disease (IgA antiendomysial antibody, IgA antitissue transglutaminase antibody, and IgA antigliadin antibody) were all negative. Serum level of IgA was markedly low. A diagnosis of atypical celiac disease with severe selective IgA deficiency was made. After the institution of a gluten-free diet (GFD), his general condition as well as anemia improved. Histological recovery was documented on repeat duodenal biopsy 6 months after GFD.