Plummer-Vinson syndrome
1010562
224267977
2008-07-08T02:09:26Z
Tdvorak
265421
/* Complications */ added hypopharynx
{{Infobox_Disease |
Name = Plummer-Vinson syndrome |
Image = |
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 [[biological tissue|tissue]] growing in the [[throat]] ([[esophageal web]]s).<ref name="pmid16978405">{{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}}</ref>
==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, [[Genetics|genetic]] factors and [[nutrition|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]].<ref name="WhoNamedIt">{{WhoNamedIt|synd|1777}}</ref><ref>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.</ref><ref>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.</ref>
It is also sometimes called Kelly-Paterson syndrome, after [[Adam Brown-Kelly]] and [[Donald Ross Paterson]].<ref name="WhoNamedIt">{{WhoNamedIt|synd|1777}}</ref><ref>A. B. Kelly. Spasm at the entrance of the esophagus. The Journal of Laryngology, Rhinology, and Otology, London, 1919, 34: 285-289.</ref><ref>D. R. Paterson. A clinical type of dysphagia. The Journal of Laryngology, Rhinology, and Otology, London, 1919, 24: 289-291.</ref>
==Treatment==
Treatment is primarily aimed at correcting the iron-deficiency anemia. Patients with PVS should receive [[iron]] supplementation in their [[Diet (nutrition)|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.<ref name="pmid18031398">{{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}}</ref>
==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 and hypopharynx.
==Prevention==
Good nutrition with adequate intake of iron may prevent this disorder.
==References==
<references/>
* [http://www.nlm.nih.gov/medlineplus/ency/article/001158.htm 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==
* {{Chorus|00325}}
{{Hematology}}
[[Category:Blood disorders]]
[[Category:Gastroenterology]]
[[de:Plummer-Vinson-Syndrom]]
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[[pl:Zespół Plummera-Vinsona]]