Plummer-Vinson syndrome

Plummer-Vinson syndrome (also called Paterson-Kelly syndrome) results from chronic iron deficiency, which leads to atrophic changes in mucosal tissues, including the formation of thin, web-like membranes in the upper esophagus (esophageal webs). This causes progressive dysphagia, typically for solids more than liquids, due to mechanical obstruction. Patients also present with glossitis (a smooth, beefy-red tongue due to papillary atrophy), angular stomatitis (cheilosis), koilonychia (spoon-shaped nails), and other signs of iron deficiency anemia such as fatigue and pallor. The exact pathophysiology linking iron deficiency to web formation is not fully understood but may involve impaired epithelial integrity and cell-mediated changes in the absence of adequate iron-dependent enzymes. Diagnosis is typically made via barium swallow study or endoscopy demonstrating esophageal webs, combined with laboratory evidence of iron deficiency anemia (low ferritin, low serum iron, high TIBC, microcytic hypochromic RBCs). Treatment focuses on iron supplementation, which often resolves the anemia and may improve or resolve the webs; esophageal dilation may be needed for significant dysphagia. Clinically, this syndrome is significant because it is associated with an increased risk of developing squamous cell carcinoma of the esophagus and hypopharynx, thought to be due to chronic mucosal irritation and atrophic changes from long-standing iron deficiency. It is more common in women, particularly in the fourth to seventh decades of life, and has been linked to Scandinavian and Northern European descent in some studies, though it can occur in any population, especially with chronic malnutrition or iron-deficient states. On board exams, key associations include the triad (anemia, esophageal web, dysphagia) plus glossitis, and the increased malignancy risk should prompt surveillance in affected patients.