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Plummer–Vinson syndrome (PVS), also called Paterson–Brown–Kelly syndrome or sideropenic dysphagia, is a rare disease characterized by difficulty in 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 PVS 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 postmenopausal women. Its identification and follow-up is considered relevant due to increased risk of squamous cell carcinomas of the esophagus and pharynx.
PVS sufferers often complain of 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:
Serial contrasted gastrointestinal radiography or upper gastrointestinal endoscopy may reveal the web in the esophagus. Blood tests show 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.
The cause of PVS is unknown; however, genetic factors and nutritional deficiencies may play a role. It is more common in women, particularly in middle age (peak age is over 50). In these patients, esophageal squamous cell carcinoma risk is increased; therefore, it is considered a premalignant process.
The condition is associated with koilonychia, glossitis, inflammation of the lips (cheilitis), and splenomegaly. Esophageal web in Plummer-Vinson syndrome is found at upper end of esophagus(post cricoid region) and Schatzki ring may be found at the lower end of esophagus.
The diagnosis is based on the evidence of iron-deficiency anemia and one or more esophageal webs in a patient with postcricoid dysphagia. Esophageal webs can be detected by barium swallow X-ray but the best way for demonstration is the videofluoroscopy. Webs are also detectable by upper gastrointestinal endoscopy. They appear smooth, thin, and gray with eccentric or central lumen. The webs typically occur in the proximal part of the esophagus and may be missed and accidentally ruptured unless the endoscope is introduced under direct visualization.
The esophageal webs, which can also occur in the absence of anemia and Plummer-Vinson syndrome, are characterized by one or more thin horizontal membranes consisting of squamous epithelium and submucosa. They usually protrude from the anterior wall, extending laterally but not to the posterior wall, which means that they rarely encircle the lumen.
Good nutrition with adequate intake of iron may prevent this disorder. Good nutrition should also include balanced diet and exercise.
Treatment is primarily aimed at correcting the iron-deficiency anemia. Patients with PVS should receive iron supplementation in their diet. This may improve dysphagia and pain.If not, the web can be dilated during upper endoscopy to allow normal swallowing and passage of food.
There is risk of perforation of the esophagus with the use of dilators for treatment. Furthermore, it is one of the risk factors for developing squamous cell carcinoma of the oral cavity, esophagus, and hypopharynx.
The disease is named after two Americans: the physician Henry Stanley Plummer and the surgeon Porter Paisley Vinson.It is occasionally known as Kelly-Paterson syndrome in the UK, after Derek Brown-Kelly and Donald Ross Paterson. However, Plummer–Vinson syndrome is still the preferred term.
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