Plummer-Vinson Syndrome

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Synonyms: Paterson-Brown Kelly syndrome, Paterson-Brown-Kelly syndrome, Waldenström-Kjellberg syndrome, sideropenic dysphagia, sideropenic nasopharyngopathy.

Plummer-Vinson syndrome (PVS) is a triad of microcytic hypochromic anaemia (iron deficiency), atrophic glossitis, and oesophageal webs or strictures.[1] A description of this syndrome was published by Henry Plummer in Chicago in 1911 and also by Porter Vinson in Philadelphia in 1919. Donald Ross Paterson and Adam Brown Kelly published independently of each other in London in 1919.[2]

PVS most often affects middle-aged women (this may be related to a propensity for iron deficiency), although it has also been recognised presenting in a child or older adults.[3, 4] The web (containing mucosa and submucosa) occurs at the anterior post-cricoid area of the upper oesophagus.[5]

It is a rare condition and much of the literature is case reports of one or a few individuals rather than large series. Figures for incidence and prevalence are not available but it is becoming rarer in the developed world, probably because dietary inadequacy is becoming less common.[6]

A high prevalence of PVS was reported in Sweden in the early 20th century. This reduced after the introduction of iron supplementation in food.[7]

PVS appears to be more common in the Indian subcontinent than in Western countries. PVS is common in males as well as females in the Indian subcontinent.[8, 9]

Risk factors

Iron deficiency - this is related to poor diet or blood loss.[10]

The presentation is usually with painless, intermittent dysphagia. It tends to be with solid foods but, if untreated, may progress to soft foods and even liquids. As with other causes of oesophageal strictures, webs and rings, the patient is able to give a good indication of the level of obstruction. In this condition the patient indicates the upper oesophagus. Dysphagia may lead to weight loss. This should be regarded as a sign of danger.

There may also be features of iron-deficiency anaemia like lethargy, tiredness and shortness of breath on exertion.

The features that appear on examination are those associated with iron deficiency. There may be pallor and even tachycardia if anaemia is marked. There may be koilonychia (spoon-shaped nails), angular cheilitis and glossitis.

  • FBC will show a microcytic, hypochromic anaemia. Ferritin is low.
  • Barium swallow may show the web. This may need to be enhanced with videofluoroscopy.
  • Biopsy may be required if malignancy is suspected clinically.

Various disorders have been known to associate PVS, such as coeliac disease, inflammatory bowel disease, pernicious anaemia, thyroid disease, Sjögren's syndrome and rheumatoid arthritis.

  • Iron replacement can almost invariably be achieved by oral means. There is rarely any need for parenteral iron. Supplements may be needed long-term because after correction it is important to maintain a normal iron status. Causes of blood loss like menorrhagia may require attention.
  • Regular endoscopic surveillance is required to detect transformation into squamous cell carcinoma.[14]
  • Endoscopic dilatation or argon plasma coagulation therapy of the oesophageal web is occasionally required in cases of persistent dysphagia.[15]

Post-cricoid carcinoma or carcinoma of the oesophagus may develop.[10]

Correction of iron deficiency will correct the condition if malignancy has not yet occurred. Prevention of iron deficiency prevents the disease.

  • Henry Plummer (1874-1937) was an American internist. He was a professor at the Medical School of Minnesota. He published: 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.
  • Porter Paisley Vinson (1890-1959) was an American surgeon at the Mayo Clinic. He published: A case of cardiospasm with dilatation and angulation of the esophagus. Medical Clinics of North America, Philadelphia, PA., 1919, 3: 623-627.
  • Born in Inverness, Donald Ross Paterson (1863-1939) was an otolaryngologist. He worked in Cardiff and published: A clinical type of dysphagia. The Journal of Laryngology, Rhinology and Otology, London, 1919, 24: 289-291.
  • Adam Brown Kelly (1865-1941) graduated from Glasgow but worked as an ENT surgeon in London, Berlin and Vienna. He published: Spasm at the entrance of the esophagus. The Journal of Laryngology, Rhinology and Otology, London, 1919, 34: 285-289.

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Further reading and references

  1. Samad A, Mohan N, Balaji RV, et al; Oral manifestations of plummer-vinson syndrome: a classic report with literature review. J Int Oral Health. 2015 Mar7(3):68-71.

  2. Plummer-Vinson Syndrome;

  3. de la Rosa Mordan Y, Rodrigo Garcia G, Miranda Cid C, et al; [Dysphagia and Anemia. Plummer-Vinson syndrome]. An Pediatr (Engl Ed). 2019 May90(5):321-322. doi: 10.1016/j.anpedi.2018.03.019.

  4. Harmouch F, Liaquat H, Chaput KJ, et al; Plummer-Vinson Syndrome: A Rare Cause of Dysphagia in an Octogenarian. Am J Case Rep. 2021 Apr 2422:e929899. doi: 10.12659/AJCR.929899.

  5. Gude D, Bansal D, Malu A; Revisiting Plummer Vinson syndrome. Ann Med Health Sci Res. 2013 Jan3(1):119-21. doi: 10.4103/2141-9248.109476.

  6. Hefaiedh R, Boutreaa Y, Ouakaa-Kchaou A, et al; Plummer-Vinson syndrome. Tunis Med. 2010 Oct88(10):721-4.

  7. Richie JP Jr, Kleinman W, Marina P, et al; Blood iron, glutathione, and micronutrient levels and the risk of oral cancer. Nutr Cancer. 200860(4):474-82. doi: 10.1080/01635580801956477.

  8. Bakshi SS; Plummer Vinson syndrome - is it common in males? Arq Gastroenterol. 2015 Jul-Sep52(3):250-2. doi: 10.1590/S0004-28032015000300018.

  9. Goel A, Bakshi SS, Soni N, et al; Iron deficiency anemia and Plummer-Vinson syndrome: current insights. J Blood Med. 2017 Oct 198:175-184. doi: 10.2147/JBM.S127801. eCollection 2017.

  10. Verma S, Mukherjee S; Plummer Vinson Syndrome.

  11. Liu CC, Lin YS; Plummer-Vinson Syndrome. Ear Nose Throat J. 2019 Jun98(5):259-260. doi: 10.1177/0145561319850412.

  12. Phatak S, Redkar N, Patil MA, et al; Plummer-Vinson syndrome. BMJ Case Rep. 2012 Jul 252012. pii: bcr2012006403. doi: 10.1136/bcr-2012-006403.

  13. Lo KB, Albano J, Sandhu N, et al; Plummer-Vinson syndrome: improving outcomes with a multidisciplinary approach. J Multidiscip Healthc. 2019 Jun 1912:471-477. doi: 10.2147/JMDH.S180410. eCollection 2019.

  14. Goh WGW, Ng DCY, Ng JX, et al; Paterson-Brown Kelly Syndrome (also commonly known as Plummer-Vinson Syndrome). Clin Case Rep. 2020 Jul 208(11):2316-2317. doi: 10.1002/ccr3.3127. eCollection 2020 Nov.

  15. Hirose T, Funasaka K, Furukawa K, et al; Plummer-Vinson Syndrome with Esophageal Web Formation in which Detailed Endoscopic Images Were Obtained. Intern Med. 2019 Mar 1558(6):785-789. doi: 10.2169/internalmedicine.1628-18. Epub 2018 Nov 19.