Plummer–Vinson syndrome (also known as Paterson–Kelly syndrome
or Paterson–Brown-Kelly syndrome in the
UK) is a
rare disease
A rare disease is any disease that affects a small percentage of the population. In some parts of the world, the term orphan disease describes a rare disease whose rarity results in little or no funding or research for treatments, without financi ...
characterized by
dysphagia
Dysphagia is difficulty in swallowing. Although classified under " symptoms and signs" in ICD-10, in some contexts it is classified as a condition in its own right.
It may be a sensation that suggests difficulty in the passage of solids or l ...
(difficulty swallowing),
iron-deficiency anemia
Iron-deficiency anemia is anemia caused by a iron deficiency, lack of iron. Anemia is defined as a decrease in the number of red blood cells or the amount of hemoglobin in the blood. When onset is slow, symptoms are often vague such as Fatigue ( ...
,
atrophic glossitis
Glossitis can mean soreness of the tongue, or more usually inflammation with depapillation of the dorsal surface of the tongue (loss of the lingual papillae), leaving a smooth and erythematous (reddened) surface, (sometimes specifically termed atro ...
(inflammation of the tongue),
angular cheilitis
Angular cheilitis (AC) is inflammation of one or both corners of the mouth. Often the corners are red with skin breakdown and crusting. It can also be itchy or painful. The condition can last for days to years. Angular cheilitis is a type of ch ...
or
cheilosis (crackings at the corners of the mouth, respectively associated or not with inflammation), and
upper esophageal webs (thin membranes in the esophagus that can cause obstruction).
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.
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
Squamous-cell carcinoma (SCC), also known as epidermoid carcinoma, comprises a number of different types of cancer that begin in squamous cells. These cells form on the surface of the skin, on the lining of hollow organs in the body, and on the ...
of the
esophagus
The esophagus (American English), oesophagus (British English), or œsophagus (Œ, archaic spelling) (American and British English spelling differences#ae and oe, see spelling difference) all ; : ((o)e)(œ)sophagi or ((o)e)(œ)sophaguses), c ...
and
pharynx
The pharynx (: pharynges) is the part of the throat behind the human mouth, mouth and nasal cavity, and above the esophagus and trachea (the tubes going down to the stomach and the lungs respectively). It is found in vertebrates and invertebrates ...
.
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
Dysphagia is difficulty in swallowing. Although classified under " symptoms and signs" in ICD-10, in some contexts it is classified as a condition in its own right.
It may be a sensation that suggests difficulty in the passage of solids or l ...
(difficulty swallowing) and, even,
Odynophagia
Odynophagia is pain when swallowing. The pain may be felt in the mouth or throat and can occur with or without difficulty swallowing. The pain may be described as an ache, burning sensation, or occasionally a stabbing pain that radiates to the ...
(painful swallowing);
*
Pain
Pain is a distressing feeling often caused by intense or damaging Stimulus (physiology), stimuli. The International Association for the Study of Pain defines pain as "an unpleasant sense, sensory and emotional experience associated with, or res ...
;
*
Weakness
Weakness is a symptom of many different medical conditions. The causes are many and can be divided into conditions that have true or perceived muscle weakness. True muscle weakness is a primary symptom of a variety of skeletal muscle diseases, ...
;
*
Atrophic glossitis
Glossitis can mean soreness of the tongue, or more usually inflammation with depapillation of the dorsal surface of the tongue (loss of the lingual papillae), leaving a smooth and erythematous (reddened) surface, (sometimes specifically termed atro ...
(smooth tongue);
*
Angular cheilitis
Angular cheilitis (AC) is inflammation of one or both corners of the mouth. Often the corners are red with skin breakdown and crusting. It can also be itchy or painful. The condition can last for days to years. Angular cheilitis is a type of ch ...
or
cheilosis (crackings at the corners of the mouth, respectively associated or not with inflammation);
*
Koilonychia (abnormally thin or spoon-shaped nails);
*
Splenomegaly
Splenomegaly is an enlargement of the spleen. The spleen usually lies in the left upper quadrant (LUQ) of the human abdomen. Splenomegaly is one of the four cardinal signs of ''hypersplenism'' which include: some reduction in number of circulat ...
(enlarged spleen);
*
Upper esophageal webs (located in the post-cricoid region, contrasting with
Schatzki rings found at the lower end of the
esophagus
The esophagus (American English), oesophagus (British English), or œsophagus (Œ, archaic spelling) (American and British English spelling differences#ae and oe, see spelling difference) all ; : ((o)e)(œ)sophagi or ((o)e)(œ)sophaguses), c ...
).
Serial contrast gastrointestinal
radiography
Radiography is an imaging technology, imaging technique using X-rays, gamma rays, or similar ionizing radiation and non-ionizing radiation to view the internal form of an object. Applications of radiography include medical ("diagnostic" radiog ...
or upper-gastrointestinal
endoscopy
An endoscopy is a procedure used in medicine to look inside the body. The endoscopy procedure uses an endoscope to examine the interior of a hollow organ or cavity of the body. Unlike many other medical imaging techniques, endoscopes are insert ...
may reveal the presence of
esophageal webs.
Blood tests
A blood test is a laboratory analysis performed on a blood sample that is usually extracted from a vein in the arm using a hypodermic needle, or via fingerprick. Multiple tests for specific blood components, such as a glucose test or a cho ...
typically show hypochromic
microcytic anemia, consistent with iron-deficiency anemia. A biopsy of the affected tongue mucosa usually reveals
epithelial atrophy (shrinking) and varying degrees of submucosal chronic inflammation. Epithelial
atypia
Atypia (from Greek language, Greek, ''a'' + ''typos'', without type; a condition of being irregular or nonstandard) is a histopathology, histopathologic term for a structural abnormality in a Cell (biology), cell, i.e. it is used to describe atypi ...
or
dysplasia
Dysplasia is any of various types of abnormal growth or development of cells (microscopic scale) or organs (macroscopic scale), and the abnormal histology or anatomical structure(s) resulting from such growth. Dysplasias on a mainly microscopic ...
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.
Causes
The cause of Plummer–Vinson syndrome is unknown; however, both
genetic factors and
nutritional deficiencies
Malnutrition occurs when an organism gets too few or too many nutrients, resulting in health problems. Specifically, it is a Deficiency (medicine), deficiency, excess, or imbalance of energy, protein and Vitamin deficiency, other nutrients whic ...
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.
Diagnosis
The following clinical presentations may be indicative of Plummer–Vinson syndrome and may be used in its diagnosis:
# 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.
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.
Prevention
Maintaining good nutrition with adequate iron intake may help prevent this disorder. A balanced diet, combined with regular exercise, is also recommended.
Treatment

Treatment for Plummer–Vinson syndrome primarily focuses on correcting the underlying
iron-deficiency anemia
Iron-deficiency anemia is anemia caused by a iron deficiency, lack of iron. Anemia is defined as a decrease in the number of red blood cells or the amount of hemoglobin in the blood. When onset is slow, symptoms are often vague such as Fatigue ( ...
. Patients should receive
iron
Iron is a chemical element; it has symbol Fe () and atomic number 26. It is a metal that belongs to the first transition series and group 8 of the periodic table. It is, by mass, the most common element on Earth, forming much of Earth's o ...
supplementation as part of their
diet, which may alleviate symptoms such as dysphagia and pain.
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.
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).
Plummer–Vinson syndrome is recognized as a risk factor for developing
squamous cell carcinoma
Squamous-cell carcinoma (SCC), also known as epidermoid carcinoma, comprises a number of different types of cancer that begin in squamous cells. These cells form on the surface of the skin, on the lining of hollow organs in the body, and on the ...
of the oral cavity, esophagus, and hypopharynx. The risk of esophageal
squamous cell carcinoma
Squamous-cell carcinoma (SCC), also known as epidermoid carcinoma, comprises a number of different types of cancer that begin in squamous cells. These cells form on the surface of the skin, on the lining of hollow organs in the body, and on the ...
is also increased,
and therefore, the syndrome is considered a
premalignant condition.
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.
However, it is very rarely observed in African countries, despite the relatively high prevalence of iron deficiency there.
History
The disease is named after two Americans working at the
Mayo Clinic
Mayo Clinic () is a Nonprofit organization, private American Academic health science centre, academic Medical centers in the United States, medical center focused on integrated health care, healthcare, Mayo Clinic College of Medicine and Science ...
—physician
Henry Stanley Plummer
Henry Stanley Plummer ( – ) was an American internist and endocrinologist. He, along with William James Mayo, William Mayo, Charles Horace Mayo, Charles Mayo, Augustus Stinchfield, E. Starr Judd, Christopher Graham, and Donald Balfour founded ...
and surgeon
Porter Paisley Vinson.
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.
Despite this, "Plummer–Vinson syndrome" remains the most commonly used name.
See also
*
List of hematologic conditions
References
External links
*
{{DEFAULTSORT:Plummer-Vinson Syndrome
Nutritional anemias
Esophagus disorders
Syndromes affecting blood
Rare diseases
Syndromes affecting the gastrointestinal tract