|Classification and external resources|
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.
Nowadays, this syndrome has become extremely rare. 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.
- Dysphagia (difficulty in swallowing)
- Odynophagia (painful swallowing)
- Atrophic glossitis
- Angular stomatitis
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.
Causes and associated conditions
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 Shatzki ring may be found at the lower end of esophagus.
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.
Good nutrition with adequate intake of iron may prevent this disorder.
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