Plummer-Vinson syndrome
| Plummer-Vinson syndrome | |
| ICD-10 | D50.1 |
|---|---|
| ICD-9 | 280.8 |
| DiseasesDB | 10134 |
| MedlinePlus | 001158 |
| eMedicine | med/3431 |
| MeSH | D011004 |
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Overview
The Plummer-Vinson syndrome, also called Paterson-Brown-Kelly syndrome or sideropenic dysphagia is a disorder linked to severe, long-term iron deficiency anemia, which causes swallowing difficulty (dysphagia) due to web-like membranes of tissue growing in the throat (esophageal webs). [1]
Presentation
P-VS sufferers often complain of a burning sensation with the tongue and oral mucosa, and atrophy of lingual papillae produces a smooth, shiny red tongue dorsum.
Causes and associated conditions
The cause of Plummer-Vinson syndrome is unknown; however, genetic factors and nutritional deficiencies may play a role.
Women are at higher risk than men, particularly in middle age. In these patients, esophageal squamous cell carcinoma risk is increased; therefore, it is considered a premalignant process.
Eponym
The disease is named after two Americans, the physician Henry Stanley Plummer, and the surgeon Porter Paisley Vinson. [2][3][4]
It is also sometimes called "Kelly-Paterson syndrome", after Adam Brown-Kelly and Donald Ross Paterson.[2][5][6]
Symptoms
- Dysphagia (difficulty in swallowing)
- Pain
- Weakness
- Odynophagia
- Atrophic Glossitis
- Angular stomatitis
Signs and tests
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 condition is associated with koilonychia, glossitis, cheilitis, and splenomegaly.
Treatment
Treatment is primarily aimed at correcting the iron-deficiency anemia. Patients with Plummer-Vinson syndrome 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.[7]
Prognosis
Patients generally respond well to treatment. Iron supplementation usually resolves the anemia, and corrects the glossodynia (tongue pain).
Complications
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 oesophagus.
Prevention
Good nutrition with adequate intake of iron may prevent this disorder.
References
- ↑ Novacek G (2006). "Plummer-Vinson syndrome". Orphanet J Rare Dis. 1: 36. doi:10.1186/1750-1172-1-36. PMID 16978405.
- ↑ 2.0 2.1 Template:WhoNamedIt
- ↑ H. S. Plummer. Diffuse dilatation of the esophagus without anatomic stenosis (cardiospasm). A report of ninety-one cases. Journal of the American Medical Association, Chicago, 1912, 58: 2013-2015.
- ↑ P. P. Vinson. A case of cardiospasm with dilatation and angulation of the esophagus. Medical Clinics of North America, Philadelphia, PA., 1919, 3: 623-627.
- ↑ A. B. Kelly. Spasm at the entrance of the esophagus. The Journal of Laryngology, Rhinology, and Otology, London, 1919, 34: 285-289.
- ↑ D. R. Paterson. A clinical type of dysphagia. The Journal of Laryngology, Rhinology, and Otology, London, 1919, 24: 289-291.
- ↑ Enomoto M, Kohmoto M, Arafa UA; et al. (2007). "Plummer-Vinson syndrome successfully treated by endoscopic dilatation". J. Gastroenterol. Hepatol. 22 (12): 2348–51. doi:10.1111/j.1440-1746.2006.03430.x. PMID 18031398.
Additional Resources
- Plummer-Vynson Syndrome. MedlinePlus Medical Encyclopedia. US Federal Government public domain. Update Date: 1/2/2003. By: Jenifer K. Lehrer, M.D., Department of Gastroenterology, Graduate Hospital, Philadelphia, PA. Review provided by VeriMed Healthcare Network.
External links
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