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]] [[fr:Syndrome de Plummer-Vinson]] [[pl:Zespół Plummera-Vinsona]]