Dysphagia is the most prominent symptom in patients with PVS and is usually painless, intermittent to progressive, limited to solid food and sometimes associated with weight loss [18]. The presence of esophageal webs can also manifest with severe choking spells or aspiration. Iron deficiency is exhibited with symptoms typical for anemia, such as weakness, fatigue, dyspnea and tachycardia. Other manifestations of iron deficiency anemia are angular cheilitis, glossitis, koilonychia (spooned nails) and pallor [3] [19]. Patients with PVS can also present very uncommonly with splenomegaly, loss of teeth and an enlarged, nodular thyroid [3].

• Anemia

  Iron therapy is the cornerstone for the treatment of iron deficiency anemia and is necessary for the resolution of anemia symptoms that accompany the disorder. [symptoma.com]

  Absence of iron overload, no hemolysis, no history suggestive of lead exposure, use of isoniazid or any chronic diseases make the alternative diagnoses such as sideroblastic anemias, thalassemias, warm antibody autoimmune hemolytic anemia, anemia of chronic [amhsr.org]

  Biologically, iron deficiency anemia is very common in patients, up to 80% of cases [8]. Hyposideremia without anemia can be found in some patients. Some authors associate SPV with sideropenia more than with anemia [7]. [scirp.org]

  Discussion Plummer Vinson Syndrome is a triad of symptoms including iron deficiency anemia, post cricoid dysphagia and esophageal webs. [article.sapub.org]

• Fatigue

  This is a case of a middle aged lady who presented with fatigue and was diagnosed to have iron deficiency anaemia. She did not have any complaints of dysphagia. On endoscopic evaluation she was found to have post cricoid web and haemorrhoids. [nepjol.info]

  Iron deficiency is exhibited with symptoms typical for anemia, such as weakness, fatigue, dyspnea and tachycardia. Other manifestations of iron deficiency anemia are angular cheilitis, glossitis, koilonychia (spooned nails) and pallor. [symptoma.com]

  This is a case of 75 year old female who presented with severe fatigue secondary to grade III dysphagia and found to have IDA. Esophagogastroduodenoscopy showed an esophageal stricture and a large hyperplastic gastric polyp. [article.sapub.org]

  Symptoms resulting from anemia such as pallor, fatigue and weakness may dominate the clinical picture. In our case pallor, fatigue and dysphagia were the presenting symptoms past 2 years. [crimsonpublishers.com]

  Symptoms resulting from anemia (weakness, pallor, fatigue, tachycardia) may dominate the clinical picture. Additional features are glossitis, angular cheilitis and koilonychia. Enlargement of the spleen and thyroid may also be observed. [orpha.net]

• Weight Loss

  His general condition was altered with conjunctival pallor, weight loss (current weight, 50 kg, for a height of 1.62 m) and WHO performance status at 3. [scirp.org]

  Over the past 2 years, she had an unintentional 40-lb weight loss, which she attributed to decreased appetite. [consultant360.com]

  The dysphagia is usually painless and intermittent or progressive over years, limited to solids and sometimes associated with weight loss. Symptoms resulting from anemia (weakness, pallor, fatigue, tachycardia) may dominate the clinical picture. [orpha.net]

  The patient had a 4-year history of severe iron deficiency anemia, a 2-year history of progressive dysphagia and weight loss, and a greater than 90% benign upper esophageal stricture. [ncbi.nlm.nih.gov]

  She had also experienced an unintentional weight loss of 4.5 kg in the last year. [amjcaserep.com]

• Surgical Procedure

  The complications of surgical procedures such as dilatations and anoplasty make it a formidable treatment challenge. [springerplus.springeropen.com]

  procedures such as collagen injections (or other facial fillers such as autologous fat or crosslinked hyaluronic acid) are used in an attempt to restore the normal facial contour.[2][4] Other measures which seek to reverse the local factors that may [en.wikipedia.org]

• Nail Abnormality

  Symptoms Difficulty swallowing Weakness Signs and tests You may develop skin and nail abnormalities thatyour doctor can see during an exam. Upper GI series or upper endoscopy may show the abnormal tissue in the food pipe. [indiatoday.in]

• Pharyngitis

  Key words: Anemia, Dysphagia, Esophageal and pharyngeal carcinoma Advertisement American Journal of Preventive Medicine and Public Health SUBMIT YOUR ARTICLE NOW [scopemed.org]

  If left untreated, progressive dysphagia can supervene and the patient is at risk of developing pharyngeal or esophageal squamous-cell carcinoma. [bibliomed.org]

  Deficiency of iron-dependent oxidative enzymes causes gradual degradation of the pharyngeal muscles which lead to mucosal atrophy and formation of webs. [ncbi.nlm.nih.gov]

  Patients need to be on annual surveillance EGD due to risk for esophageal or pharyngeal malignancy. [insights.ovid.com]

• Cough

  […] esophageal webs (eccentric and often anterior), and iron deficiency anemia Associated conditions autoimmune disease Epidemiology Most commonly observed in postmenopausal women Rare ETIOLOGY Pathogenesis unknown Presentation Symptoms dysphagia to solid foods cough [step2.medbullets.com]

  Several extraesophageal manifestations of the disease are well recognized.Those include laryngitis, cough,spectrum of injury includes esophagitis and strictures.[5]. [explainmedicine.com]

  Etiology unknown Epidemiology most commonly observed in elderly woman associated with chronic iron-deficiency anemia Patients at increased risk of developing squamous cell carcinoma of the esophagus Presentation Symptoms difficulty swallowing chronic cough [medbullets.com]

  Patient denied any cough, chest pain, light-headedness, palpitation, orthopnea or PND. She reported difficulty swallowing solid food for 3 months. She had no difficulty swallowing liquids and she would either blend her food or eat soft food. [article.sapub.org]

  The patient also had worsening coughing episodes for the last week. She also had dysphagia to both solids and liquids that had progressed over the last 6 months. [karger.com]

• Exertional Dyspnea

  dyspnea, weakness, palpitations and so on, and any one or a combination of these symptoms may bring the patient to the physician. [dovepress.com]

• Dysphagia

  Patient was lost to follow up until 6 months when patient presented again with complaints of non-resolving dysphagia and that the dysphagia progressed to liquids. [amhsr.org]

  She did not have any complaints of dysphagia. On endoscopic evaluation she was found to have post cricoid web and haemorrhoids. [nepjol.info]

  Dysphagia is the main symptom. It is usually painless and intermittent, limited to solids. In our patient, the dysphagia was permanent and global, probably related to the severity of the oesophageal ring. This dysphagia had a high cervical seat. [scirp.org]

  […] webs can be commonly found in patients without iron deficiency or dysphagia. [symptoma.com]

  Rarest subtype of esophageal dysphagia is cervical dysphagia. [biomedres.us]

• Odynophagia

  She complained of dysphagia and odynophagia and had cheilitis, glossitis, and hypochromic anemia. An esophageal motility study showed low amplitude of contraction and high intrabolus pressure in the esophageal body. [ncbi.nlm.nih.gov]

  Hurst), bougienage dilators (bougie passed over guidewire; Savary-Gilliard or American) and through-the-scope (balloon dilators) are the esophageal dilators commonly available.[8] Clinical spectrum of complications of esophageal dilation may range from odynophagia [amhsr.org]

  • Odynophagia. • Tachycardia. • Weight loss. • Underlying loose of connective tissue. • Atrophic glossitis. [drthindhomeopathy.com]

  Some may also have weight loss, odynophagia, retrosternal pain,dyspnea and hoarseness.The physical examination is usually unremarkable. [explainmedicine.com]

• Vomiting

  These may occur in the proximal and distal (Schatzki’s ring) esophagus. 47 years age female patient who admitted to outpatient clinics with complaints of dysphagia, fatique, nausea, vomiting and wasting. [bibliomed.org]

  […] peevish, and flabby • Heartburn, severe flatulence • Great weakness with headache Ferrum metallicum – • It is best adapted to young, weak and anaemic people • Weakness from speaking and walking • Pallor skin, flabby muscles and relaxed • Nausea and vomiting [drthindhomeopathy.com]

  She denied any nausea, vomiting, or any history of gastro esophageal reflux disease, diarrhea, constipation or melena. She admits to an increased craving for dirt and ice. She did not have any significant past medical, surgical or family history. [article.sapub.org]

  A 77-year-old woman was admitted to our hospital with complaints of dysphagia, vomiting, shortness of breath and weight loss for 1 month. [karger.com]

  Here we report a case of 38 years female admitted in general medicine female with complaints of breathlessness and tightness of chest relieving after vomiting and had history of anemia, dysphagia insidious in onset and dysphagia associated with solid [ijbcp.com]

• Solid Dysphagia

  The study presents the case of a 40 year old female patient who consulted for asthenia, adynamia, dizziness and progressive dyspnea with 10 months of evolution, evidencing intermittent solid dysphagia in the last 4 months. [scielo.edu.uy]

  At the age of 9 years old, he developed an upper solid dysphagia. Physical examination showed conjunctival pallor, angular cheilitis and glossitis. [biomedscis.com]

  Patient 2 A 3-year-old girl experienced a dysphagia to solid food associated with dyspnea. [journals.lww.com]

• Nausea

  These may occur in the proximal and distal (Schatzki’s ring) esophagus. 47 years age female patient who admitted to outpatient clinics with complaints of dysphagia, fatique, nausea, vomiting and wasting. [bibliomed.org]

  The patient was discharged on the 19th postoperative day with no remarkable surgical complications except a mild nausea and surgical wound discomfort. [wjgnet.com]

  […] children who are peevish, and flabby • Heartburn, severe flatulence • Great weakness with headache Ferrum metallicum – • It is best adapted to young, weak and anaemic people • Weakness from speaking and walking • Pallor skin, flabby muscles and relaxed • Nausea [drthindhomeopathy.com]

  She denied any nausea, vomiting, or any history of gastro esophageal reflux disease, diarrhea, constipation or melena. She admits to an increased craving for dirt and ice. She did not have any significant past medical, surgical or family history. [article.sapub.org]

• Koilonychia

  The characteristics may also include glossitis, glossopyrosis, glossodynia, angular cheilitis, koilonychia, fragility, thinning of nails, and brittle hair. [amhsr.org]

  The patient had pale conjunctivae, bilateral angular cheilitis, glossitis, and bilateral spoon-shaped nails (koilonychia). [consultant360.com]

  On examination, she had pallor (haemoglobin: 4 gm/dl) and typical koilonychia was present (Fig. 1). Plummer Vinson syndrome was suspected on the grounds that she was a middle aged female with anaemia, koilonychia and dysphagia to solids. [japi.org]

• Burning Sensation

  Its identification and follow-up is considered relevant due to increased risk of squamous cell carcinomas of the esophagus and pharynx. [1] Clinical presentation [ edit ] Patients with Plummer–Vinson syndrome often have a burning sensation with the tongue [en.wikipedia.org]

  As a result of an iron deficiency, a typical complex of symptoms develops with Plummer-Vinson syndrome: difficulty swallowing due to the changes in the oesophageal mucous membrane, angular cheilitis, a burning sensation on the tongue and nail changes [mta-dialog.de]

  Patients typically complain of a burning sensation of the oral mucosa, resulting in intolerance to dentures. Clinical findings include angular cheilitis and marked atrophy of lingual papillae producing a smooth, red appearance. [visualdx.com]

Workup in patients suspected to have PVS starts with diagnostic tests to confirm iron-deficiency anemia. These include a complete blood count (CBC), a peripheral blood smear and iron studies, such as serum iron, ferritin levels, total iron binding capacity (TIBC) and saturation percentage. Other tests are added, if needed.

Esophageal webs are usually detected with esophagograms or videofluoroscopy [3] [20]. A regular barium swallow can detect esophageal webs and is usually best performed with a lateral view. It would show thin projections of the anterior esophageal wall and the postcricoid. Sometimes these webs can be multiple. In case the barium swallow is negative or equivocal and the physician still maintains a high index of suspicion for PVS, a video swallow study is usually the next adequate diagnostic test. The video swallow test has the ability to distinguish true webs from false webs, which are usually caused by mucosal foldings and submucosal phenomena. Esophagogastroduodenoscopy is another potential diagnostic modality. It needs to be done very carefully, so as not to rupture the web. Otherwise, esophageal webs would go undetected. The web usually consists of a smooth, thin, gray mucosal membrane covered by squamous epithelium. They are present on the anterior esophageal walls of the proximal esophagus and extend laterally, but not to the posterior wall and are mostly crescent-shaped, although some are concentric [20].

Diagnosis is established by the confirmation of iron deficiency anemia in addition to the presence of dysphagia and one or multiple esophageal webs.  

• Esophageal Motility Disorder

  We report the case of a 41-year-old woman with Plummer-Vinson syndrome and an esophageal motility disorder. She complained of dysphagia and odynophagia and had cheilitis, glossitis, and hypochromic anemia. [ncbi.nlm.nih.gov]

  […] web 食道運動障害 esophageal motility disorder, motor disorders of the esophagus 食道炎 esophagitis アルカリ性逆流性食道炎 alkaline reflux esophagitis 逆流性食道炎 reflux esophagitis 腐食性食道炎 corrossive esophagitis 食道癌 carcinoma of the esophagus 食道胃接合部癌 cancer of esophagogastric [jsge.or.jp]

  This syndrome has to be differentiated from other causes of dysphagia, malignancy, strictures, esophageal burns, achalasia, esophageal motility disorders, etc. [ajim.in]

  Esophageal motility disorders such as scleroderma, achalasia cardia, diffuse esophageal spasm and nutcracker esophagus can also present with dysphagia, and the web may be an incidental finding. [dovepress.com]

• Microcytic Anemia

  anemia peripheral blood smear hypochromic and microcytic anemia iron studies iron deficiency Differential Schatzki ring distinguishing factor distal esophageal web Post-cricoid cancer distinguishing factor malignancy on histopathological exam Achalasia [step2.medbullets.com]

  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. [en.wikipedia.org]

• Anisocytosis

  Blood analysis revealed microcytic hypochromic anemia with anisocytosis, a hemoglobin level of 6 g/dL (normal, 12-14 g/dL), mean corpuscular volume of 65 fL (normal, 80-95 fL), mean corpuscular hemoglobin of 19 pg/dL (normal, 27-32 pg/dL), serum iron [mayoclinicproceedings.org]

  The peripheral blood smear showed anisocytosis and hypochromic microcytic cells. Thevenon test was negative. Abdominal ultrasound uterine myoma. [pesquisa.bvsalud.org]

  Peripheral blood smear showed microcytic hypochromic anemia with anisocytosis. Liver function and renal function tests were normal. Upper GI endoscopy showed postcricoid web [Figure 4]. The postcricoid web was dilated using Savary–Gilliard dilator. [ajim.in]

  The blood cells analysis showed low hematocrit and hemoglobin values as well as anisocytosis and hypochromic microcytosis. The iron's kinetic test corroborated iron deficiency. [scielo.org.mx]

  The peripheral smear of all the patients showed microcytic hypochromic red blood cells with anisocytosis. [scielo.br]

Treatment of PVS mostly occurs in the outpatient department. Iron therapy is the cornerstone for the treatment of iron deficiency anemia and is necessary for the resolution of anemia symptoms that accompany the disorder. Continuous iron supplementation beyond what is needed for the treatment of anemia is usually not required and has not proven effective [21].

Dysphagia can sometimes resolve with iron supplementation alone if not particularly severe, but obstructive dysphagia resulting from more advanced webs is better managed with mechanical dilation [22]. The passing of an endoscope can sometimes be sufficient in disrupting the web structure. Mechanical dilation is performed with the passage of a bougie (for example a Savary dilator) with a diameter of up to 17 mm after the endoscopic placement of a guidewire [23]. It is generally more appropriate to insert a single large dilator than to repeat the process more than once. Endoscopy is rarely performed under fluoroscopic guidance, unless the mechanical obstruction is so severe that it prevents the passage of the endoscope. Balloon dilation is not adequate, given the proximal location of the web, although reports of its use have been described when performed under fluoroscopic guidance [24] [25] [26]. Laser therapy and needle-knife electroincision are other potential therapeutic modalities, but their use is rarely required [27] [28] [23]. Surgery is reserved only for recalcitrant webs or those associated with Zenker diverticulum.

A change in dietary habits is important in the treatment of the disease. Solid food, especially meat, should be cut in small pieces and patients need to be advised to chew thoroughly and eat slowly.

Finally, because of the significant association of PVS with upper gastrointestinal carcinomas, patients need to be followed closely. A yearly surveillance with upper gastrointestinal endoscopy is recommended, although the effectiveness of this measure has not been confirmed [22].

Prognosis of PVS is excellent when it is not complicated by hypopharyngeal or esophageal carcinoma [14] [15]. The disease responds very well to a combination of iron supplementation, dietary changes and esophageal dilation. Morbidity is mostly related to dietary changes and repeated esophageal dilation in patients with recurrent dysphagia, with an increased risk of esophageal perforation. Mortality figures are uncertain, given the rarity of the disease, although it is thought that PVS has very low mortality.

Around 3 to 15% of patients with PVS ultimately develop cancers of the upper gastrointestinal tract  (pharynx and esophagus) [12] [16]. These relatively high rates necessitate frequent follow-up and supervision of patients with PVS. Recent data demonstrate a decrease in the rate of cancer of the hypopharynx among women, probably because of a decrease in the prevalence of PVS and better follow-up [17]. Prognosis for the disease worsens significantly when cancer is diagnosed.

The cause of Plummer-Vinson syndrome remains unknown, although several factors have been proposed, including iron and other nutritional deficiencies, a genetic predisposition and autoimmune mechanisms [7]. The most prominent theory attributes the syndrome to iron deficiency, given its involvement in the classic defining triad of the syndrome and the positive response of dysphagia and esophageal webs to iron supplementation.

The prevalence of PVS was considerably more elevated in the first half of the 20th century and then displayed a steep decline with the concomitant improvements in nutritional status, particularly with the addition of iron supplements to flour [8]. Nowadays, it is mostly found in northern European countries among middle-aged white women in the age range of 40 to 70 years, although cases involving children and adolescents have also been reported [9] [10]. It is worth noting that despite widespread malnutrition and iron deficiency in African populations, the disease remains very rare amongst this population[8].

The pathophysiology of PVS is unknown, although several theories have been proposed. They mostly involve iron deficiency, malnutrition, genetic predisposition and autoimmunity [7] [11].

The most prominent theory identifies iron deficiency as the causative mechanism and postulates that such a deficiency leads to a depletion of critical oxidative enzymes, ultimately resulting in atrophy of the esophageal mucosa, formation of webs and disturbances in muscle fiber involved in swallowing. The validity of this theory remains unaccepted and is based mostly on studies showing an improvement of dysphagia with iron supplementation, in addition to sparse and anecdotal reports of a positive response of impaired esophageal motility in patients with PVS to iron therapy [6] [12]. Nonetheless, other scientific work has cast doubt on this mechanism. Large population-based studies failed to find any association between dysphagia and anemia or sideropenia, while other work demonstrated that patients with esophageal webs are just as likely to be iron deficient as control subjects and that esophageal webs can be commonly found in patients without iron deficiency or dysphagia [6].

On the other hand, the association of PVS with several autoimmune conditions such as rheumatoid arthritis, pernicious anemia, celiac disease and thyroiditis has fueled speculation that PVS is an autoimmune disorder [7] [13]. One study reported that patients with PVS had significantly elevated levels of thyroid cytoplasmic autoimmune antibodies, when compared to control subjects with iron deficiency [7]. Overall, however, the autoimmune theory is not widely accepted.

Iron supplementation, either through dietary changes or the administration of ferrous preparations, can prevent the development of the disease in patients with or without iron deficiency anemia. It can also resolve the condition if malignancy has not yet developed.

Plummer-Vinson syndrome (PVS) is a rare disorder characterized by a triad of dysphagia, esophageal webs and iron deficiency anemia [1] [2] [3] [4] [5]. In the United States, it is also known as Paterson-Brown-Kelly syndrome. It is also associated with a significantly increased risk of squamous cell carcinoma of the pharynx and the esophagus. PVS is mostly prevalent among Caucasian middle-aged women with a history of chronic iron deficiency anemia [6]. The cause of PVS has not been established, but it is believed that a deficiency in iron leads to depletion of important oxidative enzymes and the development of myasthenic phenomena that are displayed by muscles that mediate swallowing, as well as mucosal and submucosal atrophic alterations. Patients with PVS usually present with dysphagia and symptoms of anemia, such as fatigue, weakness and dyspnea. Diagnosis is established by the presence of dysphagia and independent confirmation of iron deficiency anemia and esophageal webs. The latter is most commonly identified with barium swallow, X-Rays or videofluoroscopy. PVS usually responds to iron therapy although esophageal obstruction necessitates endoscopy and dilation. Close follow-up and periodic upper gastrointestinal endoscopy are recommended to monitor any development of cancer. PVS can be prevented in patients with chronic iron deficiency through iron supplementation and prognosis is excellent if the disease is not complicated by the development of esophageal or pharyngeal cancer.

Plummer-Vinson syndrome (PVS) is a rare disorder that manifests mostly among middle-aged white women suffering from chronic iron deficiency anemia. Patients with this condition typically develop webs of thin tissue that can block the upper third of the alimentary tract known as the esophagus, leading to problems in swallowing food.

The exact cause of this disorder is unknown but scientists suspect that a deficiency in iron (as well as other nutrients) and genetic causes play a major role. Iron is an important component of many biochemical reactions in our body and its deficiency is thought to impair the muscles involved in normal swallowing, as well as the tissue that lines the esophagus (esophageal mucosa). 

Patients with PVS most commonly complain of a difficulty in swallowing (dysphagia), usually regarding solid food, that is not associated with pain, as well as symptoms of anemia such as fatigue, weakness, pallor and shortness of breath. In order to diagnose the disease, the doctor will perform a number of blood tests to confirm iron deficiency anemia. Esophageal webs are best identified with a barium swallow X-Ray or a video swallow test that enables the doctor to visualize any webs of tissue limiting the patency of the esophagus.

PVS is most commonly treated with iron supplementation, although severe obstruction of the esophagus requires mechanical intervention. This is done by mechanically rupturing the esophageal webs using a dilator or an endoscope. Patients with PVS usually respond very well to treatment. Nonetheless, the major risk of PVS is the development of cancer of the esophagus and throat, which illustrates the importance of regular follow up with a doctor if diagnosed. General recommendations suggest the performance of a yearly endoscopy of the upper gastrointestinal tract to detect the development of any possible cancer. The development of PVS in patients who are iron deficient can be prevented with iron supplementation.

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