View source for Plummer–Vinson syndrome

{{Infobox medical condition (new)
| name = Plummer–Vinson syndrome
| synonyms = {{Unbulleted list|Paterson–Kelly syndrome|Paterson–Brown-Kelly syndrome|Sideropenic dysphagia<ref name="pmid16978405" />}}
| image = Angular Cheilitis 2.jpg
| caption = [[Angular stomatitis]]
| pronounce =
| field = [[Gastroenterology]], [[Hematology]]
| symptoms = [[Dysphagia]], [[iron-deficiency anemia]], [[glossitis]], [[cheilosis]], [[esophageal webs]]
| complications = [[Esophageal cancer]], [[pharyngeal cancer]]
| onset = Typically in [[perimenopausal]] women
| duration = Chronic, but treatable
| types =
| causes = Iron deficiency
| risks = Poor nutritional status, particularly iron deficiency
| diagnosis = Clinical evaluation, [[esophagogastroduodenoscopy]], [[blood tests]] (for iron deficiency)
| differential =
| prevention = Adequate iron intake, monitoring at-risk individuals
| treatment = Iron supplementation, mechanical dilation of the esophagus
| medication = Iron supplements
| prognosis = Generally good with treatment
| frequency = Rare
| deaths =
}}

'''Plummer–Vinson syndrome''' (also known as '''Paterson–Kelly syndrome'''<ref name="pmid16978405" /> or '''Paterson–Brown-Kelly syndrome''' in the [[UK]]<ref name=":1">{{Citation |last=Verma |first=Sandeep |title=Plummer-Vinson Syndrome |date=2023 |url=https://www.ncbi.nlm.nih.gov/books/NBK538306/ |work=StatPearls |access-date=2023-11-08 |place=Treasure Island (FL) |publisher=StatPearls Publishing |pmid=30855890 |last2=Mukherjee |first2=Sandeep}}</ref>) is a [[rare disease]] characterized by [[dysphagia]] (difficulty swallowing), [[iron-deficiency anemia]], [[glossitis|atrophic glossitis]] (inflammation of the tongue), [[angular cheilitis]] or [[cheilosis]] (crackings at the corners of the mouth, respectively associated or not with inflammation), and [[Esophageal web|upper esophageal webs]] (thin membranes in the esophagus that can cause obstruction).<ref name="pmid16978405">{{cite journal |last=Novacek G|first=Gottfried |title=Plummer-Vinson syndrome |journal=Orphanet Journal of Rare Diseases |volume=1 |pages=36 |year=2006 |pmid=16978405 |doi=10.1186/1750-1172-1-36 |pmc=1586011 |doi-access=free }}</ref> Treatment with [[iron supplementation]] and mechanical widening of the esophagus generally leads to excellent outcomes.

While exact epidemiological data are lacking, Plummer–Vinson syndrome has become extremely rare. The reduction in prevalence has been hypothesized to result from improvements in nutritional status and iron availability in countries where the syndrome was previously more common.<ref name="pmid16978405"/> The syndrome generally occurs in [[perimenopausal]] women. Identification and follow-up of affected individuals are important due to the increased risk of [[squamous cell carcinoma]] of the [[esophagus]] and [[pharynx]].<ref name="pmid16978405"/>

== Presentation ==
Patients with Plummer–Vinson syndrome often experience a burning sensation in the tongue and oral mucosa, and atrophy of the lingual papillae results in a smooth, shiny, red dorsum of the tongue. Common symptoms include:
* [[Dysphagia]] (difficulty swallowing) and, even, [[Odynophagia]] (painful swallowing);
* [[Pain]];
* [[Weakness]];
* [[Atrophic glossitis]] (smooth tongue);
* [[Angular cheilitis]] or [[cheilosis]] (crackings at the corners of the mouth, respectively associated or not with inflammation);
* [[Koilonychia]] (abnormally thin or spoon-shaped nails);
* [[Splenomegaly]] (enlarged spleen);
* [[Esophageal web|Upper esophageal webs]] (located in the post-cricoid region, contrasting with [[Schatzki rings]] found at the lower end of the [[esophagus]]).<ref>Sabiston 18th edition, p. 1078.</ref>

Serial contrast gastrointestinal [[radiography]] or upper-gastrointestinal [[endoscopy]] may reveal the presence of [[Esophageal web|esophageal webs]].

[[Blood tests]] typically show hypochromic [[microcytic anemia]], consistent with iron-deficiency anemia. A biopsy of the affected tongue mucosa usually reveals [[Atrophy|epithelial atrophy]] (shrinking) and varying degrees of submucosal chronic inflammation. Epithelial [[atypia]] or [[dysplasia]] may also be present. 

In some cases, the syndrome may manifest as post-cricoid malignancy, which can be detected by the absence of laryngeal crepitus. Normally, laryngeal crepitus is produced when the cricoid cartilage rubs against the vertebrae.{{citation needed|date=July 2020}}

==Causes==
The cause of Plummer–Vinson syndrome is unknown; however, both [[Genetics|genetic]] factors and [[nutritional deficiencies]] may play a role. The syndrome is more common in women, particularly those in middle age, with a peak incidence occurring in individuals over 50 years of age.<ref name=":0">{{cite web|title=Plummer-Vinson syndrome: MedlinePlus Medical Encyclopedia|url=https://www.medlineplus.gov/ency/article/001158.htm|year=2011}}</ref>

==Diagnosis==
The following clinical presentations may be indicative of Plummer–Vinson syndrome and may be used in its diagnosis:{{citation needed|date=July 2020}}

# Dizziness
# Pallor of the conjunctiva and face
# Erythematous oral mucosa with a burning sensation
# Breathlessness
# Atrophic, smooth tongue
# Peripheral [[rhagades]] (cracks or fissures) 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) are essential for confirming iron deficiency, with or without the presence of hypochromic microcytic anemia.{{citation needed|date=July 2020}}

===Imaging===
Barium esophagography and videofluoroscopy can aid in detecting esophageal webs. Esophagogastroduodenoscopy allows for the visual confirmation of these webs, which are caused by subepithelial fibrosis.{{citation needed|date=July 2020}}
[[File:Web114.jpg|thumb|Upright double contrast image showing jet phenomena from cervical esophageal web.]]
[[File:Cervical esophageal web.jpg|thumb|Right posterior oblique prone single contrast fluoroscopic image showing a circumferential thin cervical esophageal web.]]

==Prevention==
Maintaining good nutrition with adequate iron intake may help prevent this disorder. A balanced diet, combined with regular exercise, is also recommended.{{citation needed|date=July 2020}}

==Treatment==
[[File:Ascorbic-acid-from-xtal-1997-3D-balls.png|thumb|160 px|Ascorbic acid]]
Treatment for Plummer–Vinson syndrome primarily focuses on correcting the underlying [[iron-deficiency anemia]]. Patients should receive [[iron]] supplementation as part of their [[Diet (nutrition)|diet]], which may alleviate symptoms such as dysphagia and pain.<ref name="pmid16978405" /> If symptoms persist, the esophageal web can be dilated using esophageal bougies during [[upper endoscopy]], which allows for normal swallowing and the passage of food.<ref name="pmid18031398">{{cite journal |vauthors=Enomoto M, Kohmoto M, Arafa UA, etal |title=Plummer-Vinson syndrome successfully treated by endoscopic dilatation |journal=Journal of Gastroenterology and Hepatology |volume=22 |issue=12 |pages=2348–51 |year=2007 |pmid=18031398 |doi=10.1111/j.1440-1746.2006.03430.x |s2cid=33777667 |doi-access=free }}</ref> However, there is a 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).<ref name="pmid16978405" /> Plummer–Vinson syndrome is recognized as a risk factor for developing [[squamous cell carcinoma]] of the oral cavity, esophagus, and hypopharynx.<ref>{{cite web|title=Plummer-Vinson Syndrome |url=https://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0002143/|archive-url=https://web.archive.org/web/20120908111148/http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0002143/|url-status=dead|archive-date=September 8, 2012|publisher=PubMed Health|access-date=31 January 2012}}</ref> The risk of esophageal [[squamous cell carcinoma]] is also increased,<ref name="pmid16978405" /> and therefore, the syndrome is considered a [[premalignant]] condition.<ref name="Goel">{{cite journal|last1=Goel|first1=A|last2=Bakshi|first2=SS|last3=Soni|first3=N|last4=Chhavi|first4=N|date=2017|title=Iron deficiency anemia and Plummer-Vinson syndrome: current insights.|journal=Journal of Blood Medicine|volume=8|pages=175–184|doi=10.2147/JBM.S127801|pmc=5655134|pmid=29089792|doi-access=free}}</ref>

==Epidemiology==
Plummer–Vinson syndrome (PVS) is an extremely rare condition, and its exact prevalence remains unknown. While it is becoming less common in developed countries, the condition is increasingly found in developing regions, particularly in Asia.<ref name=Goel /> However, it is very rarely observed in African countries, despite the relatively high prevalence of iron deficiency there.<ref name=Goel />

== History ==
The disease is named after two Americans working at the [[Mayo Clinic]]—physician [[Henry Stanley Plummer]] and surgeon [[Porter Paisley Vinson]].<ref name="WhoNamedIt">{{WhoNamedIt|synd|1777}}</ref><ref>{{cite journal |first=H.S. |last=Plummer |title=Diffuse dilatation of the esophagus without anatomic stenosis (cardiospasm). A report of ninety-one cases |journal=Journal of the American Medical Association |date=1912 |volume=58 |pages=2013–2015 |doi=10.1001/jama.1912.04260060366003}}</ref><ref>{{cite journal |first=P.P. |last=Vinson |title=A case of cardiospasm with dilatation and angulation of the esophagus |journal=Medical Clinics of North America |location=Philadelphia, PA |date=1919 |volume=3 |pages=623–627}}</ref> In the UK, it is known as Paterson–Kelly or Paterson–Brown-Kelly syndrome, named after [[Derek Brown-Kelly]] and [[Donald Ross Paterson]], who independently described the syndrome in 1919.<ref name="pmid16978405"/><ref name=":1" /> Despite this, "Plummer–Vinson syndrome" remains the most commonly used name.<ref name="pmid16978405"/>

==See also==
* [[List of hematologic conditions]]

==References==
{{Reflist}}

== External links ==
* {{Chorus|00325}}
{{Medical resources
|  DiseasesDB      = 10134
|  ICD10           = {{ICD10|D|50|1|d|50}}
|  ICD9            = {{ICD9|280.8}}
|  ICDO            = 
|  OMIM            = 
|  MedlinePlus     = 001158
|  eMedicineSubj   = med
|  eMedicineTopic  = 3431
|  MeshID          = D011004
|  Orphanet        = 54028
}}
{{Diseases of RBCs}}
{{Digestive system diseases}}

{{DEFAULTSORT:Plummer-Vinson Syndrome}}
[[Category:Nutritional anemias]]
[[Category:Esophagus disorders]]
[[Category:Syndromes affecting blood]]
[[Category:Rare diseases]]
[[Category:Syndromes affecting the gastrointestinal tract]]