Plummer–Vinson syndrome

From Wikipedia, the free encyclopedia
  (Redirected from Plummer-Vinson syndrome)
Jump to: navigation, search
Plummer–Vinson syndrome
Classification and external resources
ICD-10 D50.1
ICD-9 280.8
DiseasesDB 10134
MedlinePlus 001158
eMedicine med/3431
MeSH D011004

Plummer–Vinson syndrome (PVS), also called Paterson–Brown–Kelly syndrome or sideropenic dysphagia, presents as a triad of postcricoid dysphagia from esophageal webs, iron deficiency anemia, and a beefy-red tongue due to atrophic glossitis.[1] It most usually occurs in postmenopausal women.

Presentation[edit]

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:

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[edit]

The cause of PVS is unknown; however, genetic factors and nutritional deficiencies may play a role. It is more common in women,[2] particularly in middle age (peak age is over 50). In these patients, esophageal squamous cell carcinoma risk is increased;[1] therefore, it is considered a premalignant process.

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

Eponym[edit]

The disease is named after two Americans: the physician Henry Stanley Plummer and the surgeon Porter Paisley Vinson.[3][4][5]

It is preferentially known as Kelly-Paterson syndrome in the UK, after Derek Brown-Kelly and Donald Ross Paterson.[3][6][7]

Treatment[edit]

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.[citation needed]

If not, the web can be dilated during upper endoscopy to allow normal swallowing and passage of food.[8]

Prognosis[edit]

Patients generally respond well to treatment. Iron supplementation usually resolves the anemia, and corrects the glossodynia (tongue pain).[citation needed]

Complications[edit]

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.[9]

Prevention[edit]

Good nutrition with adequate intake of iron may prevent this disorder.[citation needed]

See also[edit]

References[edit]

  1. ^ a b Novacek G (2006). "Plummer-Vinson syndrome". Orphanet J Rare Dis 1: 36. doi:10.1186/1750-1172-1-36. PMC 1586011. PMID 16978405. 
  2. ^ "Plummer-Vinson syndrome: MedlinePlus Medical Encyclopedia". 2011. 
  3. ^ a b synd/1777 at Who Named It?
  4. ^ 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.
  5. ^ 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.
  6. ^ A. B. Kelly. Spasm at the entrance of the esophagus. The Journal of Laryngology, Rhinology, and Otology, London, 1919, 34: 285-289.
  7. ^ D. R. Paterson. A clinical type of dysphagia. The Journal of Laryngology, Rhinology, and Otology, London, 1919, 24: 289-291.
  8. ^ 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. 
  9. ^ "Plummer-Vinson Syndrome url=http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0002143/". PubMed Health. 

External links[edit]