Plummer–Vinson syndrome is a rare disease characterized by difficulty swallowing, iron-deficiency anemia, glossitis, cheilosis and esophageal webs. Treatment with iron supplementation and mechanical widening of the esophagus generally provides an excellent outcome. While exact data about the epidemiology is unknown, this syndrome has become extremely rare. The reduction in the prevalence of Plummer–Vinson syndrome has been hypothesized to be the result of improvements in nutritional status and availability in countries where the syndrome was previously described. It generally occurs in perimenopausal women. Its identification and follow-up is considered relevant due to increased risk of squamous cell carcinomas of the esophagus and pharynx. Presentation
Patients with Plummer–Vinson syndrome often have a burning sensation with the tongue and oral mucosa, and atrophy of lingual papillae produces a smooth, shiny, red, dorsum of the tongue. Symptoms include:
Dysphagia (difficulty swallowing)
Pain
Weakness
Odynophagia (painful swallowing)
Atrophic glossitis
Angular cheilitis
Koilonychia (Abnormally thin nails, also called spoon nails)
Splenomegaly (an enlarged spleen). Upper esophageal webs (post cricoid region -  contrasts with Schatzki rings found at the lower end of esophagus).Serial contrasted gastrointestinal radiography or upper-gastrointestinal endoscopy may reveal the web in the esophagus. Blood tests demonstrate a hypochromic microcytic anemia that is consistent with an iron-deficiency anemia. Biopsy of involved mucosa typically reveals epithelial atrophy (shrinking) and varying amounts of submucosal chronic inflammation. Epithelial atypia or dysplasia may be present. It may also present as a post-cricoid malignancy which can be detected by loss of laryngeal crepitus. Laryngeal crepitus is found normally and is produced because the cricoid cartilage rubs against the vertebrae.
Esophageal squamous cell carcinoma risk is also increased; therefore, it is considered a premalignant condition. Epidemiology
PVS is an extremely rare condition, though its exact prevalence is unknown. While it is becoming less common in developed countries, the condition is increasingly found in developing countries, particularly in Asia. However, it is very rarely seen in African countries, despite the relatively high prevalence of iron deficiency. History
The disease is named after two Americans working at the Mayo Clinic -- the physician Henry Stanley Plummer and the surgeon Porter Paisley Vinson. It is occasionally known as Paterson-Kelly or Paterson-Brown Kelly syndrome in the UK, after Derek Brown-Kelly and Donald Ross Paterson. However, Plummer–Vinson syndrome is still the most commonly used name. See also
List of hematologic conditions

References
External links
00325 at CHORUS