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:
- Dysphagia (difficulty in swallowing)
- Odynophagia (painful swallowing)
- Atrophic glossitis
- Angular stomatitis
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.
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, with a peak age over 50 years. 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.
Following clinical presentations may be used in the diagnosis of this condition.
- Pallor of the conjuctiva and face
- Erythematous oral mucosa with burning sensation
- Atrophic and smooth tongue
- Peripheral rhagades around the oral cavity
The following tests are helpful in the diagnosis of Plummer–Vinson syndrome.
complete blood cell (CBC) counts, peripheral blood smears, and iron studies (e.g., serum iron, total iron-binding capacity [TIBC], ferritin, saturation percentage) to confirm iron deficiency, with or without hypochromic microcytic anemia.
Barium esophagography and videofluoroscopy will help to detect esophageal webs. Esophagogastroduodenoscopy will enable visual confirmation of esophageal webs. The webs occur due to sub-epithelial fibrosis.
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 with esophageal bougies 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 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.
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