Plummer–Vinson syndrome

Plummer–Vinson syndrome (known as Paterson–Kelly[1] or

Plummer–Vinson
Paterson–Brown-Kelly syndrome in the UK[2]) is a rare disease
syndrome
characterized by difficulty swallowing, iron-deficiency anemia,
glossitis, cheilosis and esophageal webs.[1] Treatment with iron Other Paterson–Kelly
supplementation and mechanical widening of the esophagus names syndrome
generally provides an excellent outcome. Paterson–Brown-Kelly
syndrome
While exact data about the epidemiology is unknown, this
syndrome has become extremely rare. The reduction in the Sideropenic
prevalence of Plummer–Vinson syndrome has been hypothesized dysphagia[1]
to be the result of improvements in nutritional status and
availability in countries where the syndrome was previously
described.[1] It generally occurs in perimenopausal women. Its
identification and follow-up is considered relevant due to increased
risk of squamous cell carcinomas of the esophagus and pharynx.[1]

Presentation
Angular stomatitis
Patients with Plummer–Vinson syndrome often have a burning Specialty Gastroenterology
sensation with the tongue and oral mucosa, and atrophy of lingual
papillae produces a smooth, shiny, red, dorsum of the tongue. Symptoms include:

Dysphagia (difficulty swallowing)

Pain
Weakness
Odynophagia (painful swallowing)
Atrophic glossitis
Angular cheilitis
Koilonychia (Abnormally thin nails, also called spoon nails)
Splenomegaly (an enlarged spleen).
Upper esophageal webs (post cricoid region - contrasts with Schatzki rings found at the
lower end of esophagus).[3]

Serial contrasted gastrointestinal radiography or upper-gastrointestinal endoscopy may reveal the web in the
esophagus. Blood tests demonstrate 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. It may also
present as a post-cricoid malignancy which can be detected by loss of laryngeal crepitus. Laryngeal crepitus
is found normally and is produced because the cricoid cartilage rubs against the vertebrae.

Causes
The cause of Plummer–Vinson syndrome is unknown; however, genetic factors and nutritional deficiencies
may play a role and it is more common in women,[4] particularly in middle age, with a peak age over 50
years.

Diagnosis
The following clinical presentations may be used in the diagnosis of this condition.

1. Dizziness
2. Pallor of the conjunctiva and face
3. Erythematous oral mucosa with burning sensation
4. Breathlessness
5. Atrophic and smooth tongue
6. Peripheral rhagades around the oral cavity

The following tests are helpful in the diagnosis of Plummer–Vinson syndrome.

Lab tests

Complete blood cell counts, peripheral blood smears, and iron studies (e.g., serum iron, total iron-binding
capacity, ferritin, and saturation percentage) to confirm iron deficiency, either with or without hypochromic
microcytic anemia.

Imaging

Barium esophagography and videofluoroscopy will help to detect esophageal webs.

Esophagogastroduodenoscopy will enable visual confirmation of esophageal webs. The webs occur due to
sub-epithelial fibrosis.

Prevention
Good nutrition with adequate intake of iron may prevent this disorder. Good nutrition should also include
balanced diet and exercise.

Treatment
Treatment is primarily aimed at correcting the iron-deficiency anemia. Patients with Plummer–Vinson
syndrome should receive iron supplementation in their diet. This may improve dysphagia and pain.[1] If
not, the web can be dilated with esophageal bougies during upper endoscopy to allow normal swallowing
and passage of food.[5] There is risk of perforation of the esophagus with the use of dilators for treatment.

Prognosis
Patients generally respond well to treatment. Iron supplementation
usually resolves the anemia, and corrects the glossodynia (tongue
pain).[1] Plummer–Vinson syndrome is one of the risk factors for
developing squamous cell carcinoma of the oral cavity, esophagus,
and hypopharynx.[6] Esophageal squamous cell carcinoma risk is
also increased;[1] therefore, it is considered a premalignant
condition.[7]

Epidemiology
PVS is an extremely rare condition, though its exact prevalence is
unknown. While it is becoming less common in developed
countries, the condition is increasingly found in developing
countries, particularly in Asia.[7] However, it is very rarely seen in
African countries, despite the relatively high prevalence of iron
deficiency.[7]
Upright double contrast image
History showing jet phenomennna from
cervical esophageal web.

The disease is named after two Americans working at the Mayo

Clinic—the physician Henry Stanley Plummer and the surgeon
Porter Paisley Vinson.[8][9][10] It is known as Paterson–Kelly or
Paterson–Brown-Kelly syndrome in the UK, after Derek Brown-
Kelly and Donald Ross Paterson who independently described the
syndrome in 1919.[1][2] However, Plummer–Vinson syndrome is
still the most commonly used name.[1]

See also
List of hematologic conditions

References
1. Novacek G, Gottfried (2006). "Plummer-Vinson
syndrome" (https://www.ncbi.nlm.nih.gov/pmc/articles/P
MC1586011). Orphanet Journal of Rare Diseases. 1: 36.
doi:10.1186/1750-1172-1-36 (https://doi.org/10.1186%2F
1750-1172-1-36). PMC 1586011 (https://www.ncbi.nlm.ni
h.gov/pmc/articles/PMC1586011). PMID 16978405 (http Right posterior oblique prone single
s://pubmed.ncbi.nlm.nih.gov/16978405). contrast fluoroscopic last image hold
2. Verma, Sandeep; Mukherjee, Sandeep (2023), image showing circumferential thin
"Plummer-Vinson Syndrome" (http://www.ncbi.nlm.nih.g cervical esophageal web.
ov/books/NBK538306/), StatPearls, Treasure Island
(FL): StatPearls Publishing, PMID 30855890 (https://pub
med.ncbi.nlm.nih.gov/30855890), retrieved 2023-11-08
3. Sabiston 18th edition, p. 1078.
4. "Plummer-Vinson syndrome: MedlinePlus Medical Encyclopedia" (https://www.nlm.nih.gov/
medlineplus/ency/article/001158.htm). 2011.
 5. Enomoto M, Kohmoto M, Arafa UA, et al. (2007). "Plummer-
Vinson syndrome successfully treated by endoscopic dilatation"
(https://doi.org/10.1111%2Fj.1440-1746.2006.03430.x). Journal
of Gastroenterology and Hepatology. 22 (12): 2348–51.
doi:10.1111/j.1440-1746.2006.03430.x (https://doi.org/10.1111%
2Fj.1440-1746.2006.03430.x). PMID 18031398 (https://pubmed.n
cbi.nlm.nih.gov/18031398). S2CID 33777667 (https://api.semanti
cscholar.org/CorpusID:33777667). Ascorbic-acid
6. "Plummer-Vinson Syndrome" (https://www.ncbi.nlm.nih.gov/pub
medhealth/PMH0002143/). PubMed Health. Retrieved
31 January 2012.
7. Goel, A; Bakshi, SS; Soni, N; Chhavi, N (2017). "Iron deficiency anemia and Plummer-
Vinson syndrome: current insights" (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC565513
4). Journal of Blood Medicine. 8: 175–184. doi:10.2147/JBM.S127801 (https://doi.org/10.214
7%2FJBM.S127801). PMC 5655134 (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC56551
34). PMID 29089792 (https://pubmed.ncbi.nlm.nih.gov/29089792).
8. synd/1777 (http://www.whonamedit.com/synd.cfm/1777.html) at Who Named It?
9. 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.
10. 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.

External links
00325 (http://chorus.rad.mcw.edu/doc/00325.html) at CHORUS

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