Plummer–Vinson syndrome

Presentation

Patients often report a burning sensation in the tongue and oral mucosa, with atrophy of the lingual papillae giving a smooth, shiny, red tongue surface. Symptoms include dysphagia, odynophagia, weakness, atrophic glossitis, angular cheilitis or cheilosis, koilonychia, and splenomegaly. Upper esophageal webs are located in the post-cricoid region. Serial contrast gastrointestinal radiography or upper-gastrointestinal endoscopy may reveal these webs.

Blood tests typically show hypochromic microcytic anemia. Biopsy of the tongue mucosa often shows epithelial atrophy and chronic inflammation; epithelial atypia or dysplasia may be present. In some cases, post-cricoid malignancy may manifest, detectable by absence of laryngeal crepitus.

Diagnosis

Clinical presentations that may indicate Plummer–Vinson syndrome include dizziness, pallor of the conjunctiva and face, erythematous oral mucosa with a burning sensation, breathlessness, atrophic smooth tongue, and peripheral rhagades around the oral cavity. Diagnostic tests include complete blood cell counts, peripheral blood smears, and iron studies to confirm iron deficiency. Barium esophagography and videofluoroscopy can detect esophageal webs, while esophagogastroduodenoscopy allows visual confirmation of webs caused by subepithelial fibrosis.

Did You Know?

• The syndrome is named after Henry Stanley Plummer and Porter Paisley Vinson of the Mayo Clinic; in the UK it is called Paterson–Kelly or Paterson–Brown-Kelly syndrome after Derek Brown-Kelly and Donald Ross Paterson.
• The cause is unknown, but genetic factors and nutritional deficiencies may play a role.
• The condition is very rarely observed in African countries despite the high prevalence of iron deficiency there.