Plummer–Vinson syndrome is a

rare disease A rare disease is any disease that affects a small percentage of the population. In some parts of the world, an orphan disease is a rare disease whose rarity means there is a lack of a market large enough to gain support and resources for discove ...

characterized by

difficulty swallowing 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 liqui ...

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 (me ...

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 ...

cheilosis 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 che ...

and

esophageal webs Esophageal webs are thin membranes occurring anywhere along the esophagus. Presentation Its main symptoms are pain and difficulty in swallowing (dysphagia). Esophageal webs are thin membranes of normal esophageal tissue consisting of mucosa a ...

. Treatment with iron supplementation and mechanical widening of the esophagus generally provides an excellent outcome. While exact data about the epidemiology is unknown, this syndrome has become extremely rare. The reduction in the prevalence of Plummer–Vinson syndrome has been hypothesized to be the result of improvements in nutritional status and availability in countries where the syndrome was previously described. It generally occurs in

perimenopausal Menopause, also known as the climacteric, is the time in women's lives when menstrual periods stop permanently, and they are no longer able to bear children. Menopause usually occurs between the age of 47 and 54. Medical professionals often d ...

women. Its identification and follow-up is considered relevant due to increased risk of

squamous cell carcinoma Squamous-cell carcinomas (SCCs), also known as epidermoid carcinomas, comprise 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 ...

s of the

esophagus The esophagus (American English) or oesophagus (British English; both ), non-technically known also as the food pipe or gullet, is an organ in vertebrates through which food passes, aided by peristaltic contractions, from the pharynx to the ...

and

pharynx The pharynx (plural: pharynges) is the part of the throat behind the mouth and nasal cavity, and above the oesophagus and trachea (the tubes going down to the stomach and the lungs). It is found in vertebrates and invertebrates, though its struc ...

Presentation

Patients with Plummer–Vinson syndrome often have a burning sensation with the tongue and oral mucosa, and atrophy of lingual papillae produces a smooth, shiny, red, dorsum of the tongue. 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 disease#Terminology, condition in its own right. It may be a sensation that suggests difficulty in the passag ...

(difficulty swallowing) *

Pain Pain is a distressing feeling often caused by intense or damaging stimuli. The International Association for the Study of Pain defines pain as "an unpleasant sensory and emotional experience associated with, or resembling that associated with, ...

Weakness Weakness is a symptom of a number of different 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, i ...

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 b ...

(painful swallowing) *

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 ...

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 ...

Koilonychia Koilonychia, also known as spoon nails,James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical Dermatology''. (10th ed.). Saunders. . is a nail disease that can be a sign of hypochromic anemia, especially ...

(Abnormally thin nails, also called spoon 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 circulating ...

(an enlarged spleen). * Upper esophageal webs (post cricoid region - contrasts with

Schatzki ring A Schatzki ring or Schatzki–Gary ring is a narrowing of the lower esophagus that can cause difficulty swallowing (dysphagia). The narrowing is caused by a ring of mucosal tissue (which lines the esophagus) or muscular tissue. A Schatzki ring is ...

s found at the lower end of

). Serial contrasted gastrointestinal

radiography Radiography is an 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 radiography ("diagnostic" and "therapeut ...

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 web in the esophagus.

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 cholester ...

demonstrate a hypochromic

microcytic anemia Microcytic anaemia is any of several types of anemia characterized by smaller than normal red blood cells (called microcytes). The normal mean corpuscular volume (abbreviated to MCV on full blood count results, and also known as mean cell volume) ...

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 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 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 Malnutrition occurs when an organism gets too few or too many nutrients, resulting in health problems. Specifically, it is "a deficiency, excess, or imbalance of energy, protein and other nutrients" which adversely affects the body's tissues a ...

may play a role. It is more common in women, 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. # Dizziness # Pallor of the conjunctiva and face # Erythematous oral mucosa with burning sensation # Breathlessness # Atrophic and smooth tongue # Peripheral

rhagades Rhagades are fissures, cracks, or linear scars in the skin, especially at the angles of the mouth and nose. They tend to form at areas of motion. They can be a result from bacterial infection of skin lesions. They are associated with late stages ...

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. Web114

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

. Patients with Plummer–Vinson syndrome should receive

iron Iron () is a chemical element with symbol Fe (from la, ferrum) 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, right in f ...

supplementation in their

diet Diet may refer to: Food * Diet (nutrition), the sum of the food consumed by an organism or group * Dieting, the deliberate selection of food to control body weight or nutrient intake ** Diet food, foods that aid in creating a diet for weight loss ...

. This may improve dysphagia and pain. If not, the web can be dilated with esophageal bougies during

upper endoscopy Esophagogastroduodenoscopy (EGD) or oesophagogastroduodenoscopy (OGD), also called by various other names, is a diagnostic endoscopic procedure that visualizes the upper part of the gastrointestinal tract down to the duodenum. It is considered ...

to allow normal swallowing and passage of food. 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 Burning mouth syndrome (BMS) is a burning, tingling or scalding sensation in the mouth, lasting for at least four to six months, with no underlying known dental or medical cause. No related medical sign, signs of disease are found in the mouth. ...

(tongue pain). Plummer-Vinson syndrome is one of the risk factors for developing

of the oral cavity, esophagus, and hypopharynx. Esophageal

risk is also increased; therefore, it is considered a

premalignant A precancerous condition is a condition, tumor or lesion involving abnormal cells which are associated with an increased risk of developing into cancer. Clinically, precancerous conditions encompass a variety of abnormal tissues with an increased ...

condition.

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. However, it is very rarely seen in African countries, despite the relatively high prevalence of iron deficiency.

History

The disease is named after two Americans working at the

Mayo Clinic The Mayo Clinic () is a nonprofit American academic medical center focused on integrated health care, education, and research. It employs over 4,500 physicians and scientists, along with another 58,400 administrative and allied health staff, ...

-- the

physician A physician (American English), medical practitioner (Commonwealth English), medical doctor, or simply doctor, is a health professional who practices medicine, which is concerned with promoting, maintaining or restoring health through th ...

Henry Stanley Plummer Henry Stanley Plummer ( – ) was an American internist and endocrinologist who, along with William Mayo, Charles Mayo, Augustus Stinchfield, E. Starr Judd, Christopher Graham, and Donald Balfour founded Mayo Clinic. Plummer is also immortali ...

and the surgeon Porter Paisley Vinson.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. It is occasionally known as Paterson-Kelly or Paterson-Brown Kelly syndrome in the UK, after Derek Brown-Kelly and Donald Ross Paterson. However, Plummer–Vinson syndrome is still the most commonly used name.

References

External links

* {{DEFAULTSORT:Plummer-Vinson Syndrome Nutritional anemias Esophagus disorders Syndromes affecting blood Rare diseases Syndromes affecting the gastrointestinal tract