Plummer-Vinson syndrome

Infobox_Disease
Name = Plummer-Vinson syndrome


Caption =
DiseasesDB = 10134
ICD10 = ICD10|D|50|1|d|50
ICD9 = ICD9|280.8
ICDO =
OMIM =
MedlinePlus = 001158
eMedicineSubj = med
eMedicineTopic = 3431
MeshID = D011004

Plummer-Vinson syndrome (PVS), 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).cite journal |author=Novacek G |title=Plummer-Vinson syndrome |journal=Orphanet J Rare Dis |volume=1 |issue= |pages=36 |year=2006 |pmid=16978405 |doi=10.1186/1750-1172-1-36 |url=http://www.ojrd.com/content/1//36]

Presentation

PVS 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.

Symptoms include:
* Dysphagia (difficulty in swallowing)
* Pain
* Weakness
* Odynophagia
* 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.

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.

The condition is associated with koilonychia, glossitis, cheilitis, and splenomegaly.

Eponym

The disease is named after two Americans, the physician Henry Stanley Plummer, and the surgeon Porter Paisley Vinson.WhoNamedIt|synd|1777] [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.]

It is also sometimes called Kelly-Paterson syndrome, after Adam Brown-Kelly and Donald Ross Paterson.WhoNamedIt|synd|1777] [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.]

Treatment

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.cite journal |author=Enomoto M, Kohmoto M, Arafa UA, "et al" |title=Plummer-Vinson syndrome successfully treated by endoscopic dilatation |journal=J. Gastroenterol. Hepatol. |volume=22 |issue=12 |pages=2348–51 |year=2007 |pmid=18031398 |doi=10.1111/j.1440-1746.2006.03430.x |url=http://www.blackwell-synergy.com/openurl?genre=article&sid=nlm:pubmed&issn=0815-9319&date=2007&volume=22&issue=12&spage=2348]

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 oral cavity, oesophagus and hypopharynx.

Prevention

Good nutrition with adequate intake of iron may prevent this disorder.

References

External links

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