Plummer-Vinson syndrome
Encyclopedia
Plummer–Vinson syndrome also called Paterson–Brown–Kelly syndrome or sideropenic dysphagia presents as a triad of dysphagia
(due to esophageal webs), glossitis
, and iron deficiency anemia
. It most usually occurs in postmenopausal women.
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.
factors and nutritional deficiencies
may play a role. It is more common in women, particularly in middle age. Peak age over 50 years. In these patients, esophageal squamous cell carcinoma
risk is increased; therefore, it is considered a premalignant process.
The condition is associated with koilonychia
, glossitis
, cheilitis
, and splenomegaly
.
Henry Stanley Plummer
, and the surgeon Porter Paisley Vinson
.
It is also rarely called Kelly-Paterson syndrome, after Adam Brown-Kelly and Donald Ross Paterson.
supplementation in their diet
. This may improve dysphagia and pain.
If not, the web can be dilated during upper endoscopy to allow normal swallowing and passage of food.
(tongue pain).
of the oral cavity, esophagus and hypopharynx.
Dysphagia
Dysphagia is the medical term for the symptom of difficulty in swallowing. Although classified under "symptoms and signs" in ICD-10, the term is sometimes used as a condition in its own right. Sufferers are sometimes unaware of their dysphagia....
(due to esophageal webs), glossitis
Glossitis
Glossitis is inflammation of the tongue. It causes the tongue to swell and change color. Finger-like projections on the surface of the tongue may be lost, causing the tongue to appear smooth....
, and iron deficiency anemia
Iron deficiency anemia
Iron-deficiency anemia is a common anemia that occurs when iron loss occurs, and/or the dietary intake or absorption of iron is insufficient...
. It most usually occurs in postmenopausal women.
Presentation
PVS sufferers often complain of a burning sensation with the tongue and oral mucosa, and atrophy of lingual papillae produces a smooth, shiny red tongue dorsum.Symptoms include:
- DysphagiaDysphagiaDysphagia is the medical term for the symptom of difficulty in swallowing. Although classified under "symptoms and signs" in ICD-10, the term is sometimes used as a condition in its own right. Sufferers are sometimes unaware of their dysphagia....
(difficulty in swallowing) - PainPainPain is an unpleasant sensation often caused by intense or damaging stimuli such as stubbing a toe, burning a finger, putting iodine on a cut, and bumping the "funny bone."...
- WeaknessWeaknessWeakness is a symptom represented, medically, by a number of different conditions, including: lack of muscle strength, malaise, dizziness, or fatigue. 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...
- OdynophagiaOdynophagiaOdynophagia is painful swallowing, in the mouth or esophagus. It can occur with or without dysphagia, or difficult swallowing....
(Painful swallowing) - Atrophic glossitisSmooth tongueSmooth tongue is a condition characterized by a smooth glossy tongue that is often tender/painful....
- Angular stomatitisAngular cheilitisAngular cheilitis is an inflammatory lesion at the labial commissure, or corner of the mouth, and often occurs bilaterally. The condition manifests as deep cracks or splits...
- increased risk of carcinoma
Serial contrasted gastrointestinal radiography
Radiography
Radiography is the use of X-rays to view a non-uniformly composed material such as the human body. By using the physical properties of the ray an image can be developed which displays areas of different density and composition....
or upper gastrointestinal endoscopy
Endoscopy
Endoscopy means looking inside and typically refers to looking inside the body for medical reasons using an endoscope , an instrument used to examine the interior of a hollow organ or cavity of the body. Unlike most other medical imaging devices, endoscopes are inserted directly into the organ...
may reveal the web in the esophagus. Blood tests show a hypochromic microcytic anemia
Microcytic anemia
Microcytic anemia is a generic term for any type of anemia characterized by small red blood cells. The normal mean corpuscular volume is 76-100 fL, with smaller cells as macrocytic....
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
Dysplasia
Dysplasia , is a term used in pathology to refer to an abnormality of development. This generally consists of an expansion of immature cells, with a corresponding decrease in the number and location of mature cells. Dysplasia is often indicative of an early neoplastic process...
may be present.
Causes and associated conditions
The cause of PVS is unknown; however, geneticGenetics
Genetics , a discipline of biology, is the science of genes, heredity, and variation in living organisms....
factors and nutritional deficiencies
Nutrition
Nutrition is the provision, to cells and organisms, of the materials necessary to support life. Many common health problems can be prevented or alleviated with a healthy diet....
may play a role. It is more common in women, particularly in middle age. Peak age over 50 years. In these patients, esophageal squamous cell carcinoma
Squamous cell carcinoma
Squamous cell carcinoma , occasionally rendered as "squamous-cell carcinoma", is a histologically distinct form of cancer. It arises from the uncontrolled multiplication of malignant cells deriving from epithelium, or showing particular cytological or tissue architectural characteristics of...
risk is increased; therefore, it is considered a premalignant process.
The condition is associated with koilonychia
Koilonychia
Koilonychia is a nail disease that can be a sign of hypochromic anemia, especially iron-deficiency anemia. Koilonychia literally means "spoon nails." It refers to abnormally thin nails which have lost their convexity, becoming flat or even concave in shape...
, glossitis
Glossitis
Glossitis is inflammation of the tongue. It causes the tongue to swell and change color. Finger-like projections on the surface of the tongue may be lost, causing the tongue to appear smooth....
, cheilitis
Cheilitis
Cheilitis is a medical condition involving inflammation of the lip.It is associated with many conditions, including megaloblastic anemia from vitamin B12 deficiency, iron deficiency anemia and oral candidiasis. It can also be a symptom of allergies, such as allergy to Balsam of Peru...
, and splenomegaly
Splenomegaly
Splenomegaly is an enlargement of the spleen. The spleen usually lies in the left upper quadrant of the human abdomen. It is one of the four cardinal signs of hypersplenism, some reduction in the number of circulating blood cells affecting granulocytes, erythrocytes or platelets in any...
.
Eponym
The disease is named after two Americans, the physicianPhysician
A physician is a health care provider who practices the profession of medicine, which is concerned with promoting, maintaining or restoring human health through the study, diagnosis, and treatment of disease, injury and other physical and mental impairments...
Henry Stanley Plummer
Henry Stanley Plummer
Henry Stanley Plummer, M.D. was a prominent internist and endocrinologist who, along with Drs. William Mayo, Charles Mayo, Stinchfield, E. Starr Judd, Christopher Graham, and Donald Balfour founded Mayo Clinic. Dr...
, and the surgeon Porter Paisley Vinson
Porter Paisley Vinson
Porter Paisley Vinson was a surgeon at the Mayo Clinic. He was born in 1890 to William Daniel Vinson and Lillie Helper Vinson. He was a doctor of Bronchoscopy. He married Lenore Dunlap and had three children. He died in 1959. He is best known for his contribution to medicine in the study of...
.
It is also rarely called Kelly-Paterson syndrome, after Adam Brown-Kelly and Donald Ross Paterson.
Treatment
Treatment is primarily aimed at correcting the iron-deficiency anemia. Patients with PVS should receive ironIron
Iron is a chemical element with the symbol Fe and atomic number 26. It is a metal in the first transition series. It is the most common element forming the planet Earth as a whole, forming much of Earth's outer and inner core. It is the fourth most common element in the Earth's crust...
supplementation in their diet
Diet (nutrition)
In nutrition, diet is the sum of food consumed by a person or other organism. Dietary habits are the habitual decisions an individual or culture makes when choosing what foods to eat. With the word diet, it is often implied the use of specific intake of nutrition for health or weight-management...
. This may improve dysphagia and pain.
If not, the web can be dilated during upper endoscopy to allow normal swallowing and passage of food.
Prognosis
Patients generally respond well to treatment. Iron supplementation usually resolves the anemia, and corrects the glossodyniaGlossodynia
Glossodynia or burning mouth syndrome is a condition characterized by a burning or tingling sensation on the lips, tongue, or entire mouth....
(tongue pain).
Complications
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 carcinomaSquamous cell carcinoma
Squamous cell carcinoma , occasionally rendered as "squamous-cell carcinoma", is a histologically distinct form of cancer. It arises from the uncontrolled multiplication of malignant cells deriving from epithelium, or showing particular cytological or tissue architectural characteristics of...
of the oral cavity, esophagus and hypopharynx.