|Year : 2020 | Volume
| Issue : 3 | Page : 142-144
Plummer–Vinson syndrome: A case series
ST Kavya, HR Sanjay Kumar, T Sridevi, Devapriya Rejeev
Department of General Medicine, Bangalore Medical College and Research Institute, Bengaluru, Karnataka, India
|Date of Submission||10-Sep-2019|
|Date of Decision||15-Feb-2020|
|Date of Acceptance||13-Mar-2020|
|Date of Web Publication||15-Jul-2020|
Dr. Devapriya Rejeev
Devatharu, Kuniyedath House, Marikkunnu P O, Calicut - 673 012, Kerala
Source of Support: None, Conflict of Interest: None
Plummer–Vinson syndrome (PVS), also known as Paterson–Brown–Kelly syndrome, is characterized by a triad of dysphagia, upper esophageal web, and iron deficiency anemia. It predominantly affects middle-aged females. The postcricoid webs are associated with a higher risk of squamous cell carcinoma; therefore, timely diagnosis and treatment is important. Herein, we report three cases of PVS.
Keywords: Dysphagia, iron deficiency anemia, postcricoid web
|How to cite this article:|
Kavya S T, Sanjay Kumar H R, Sridevi T, Rejeev D. Plummer–Vinson syndrome: A case series. APIK J Int Med 2020;8:142-4
|How to cite this URL:|
Kavya S T, Sanjay Kumar H R, Sridevi T, Rejeev D. Plummer–Vinson syndrome: A case series. APIK J Int Med [serial online] 2020 [cited 2020 Aug 12];8:142-4. Available from: http://www.ajim.in/text.asp?2020/8/3/142/289799
| Introduction|| |
Paterson–Brown–Kelly syndrome or Plummer–Vinson syndrome (PVS) was reported by Henry Stanley Plummer in 1912 and Porter Paisley Vinson in 1919. It is characterized by a triad of iron deficiency anemia, dysphagia, and upper esophageal webs. The disease usually affects middle-aged females. The disease usually presents with fatigue and shortness of breath which are symptoms of anemia. Dysphagia is due to upper esophageal webs and is mostly for solid foods. They can also have glossitis, koilonychia, and angular stomatitis. The exact epidemiology of the disease is not available, but this disease is extremely rare.
Herein, we report three cases of PVS, who presented with significant and long standing dysphagia, postcricoid webs, and iron deficiency anemia. Their upper gastrointestinal (GI) endoscopy revealed postcricoid webs. All three patients underwent esophageal dilatation and were started on iron supplementation.
| Case Reports|| |
A 55-year-old female presented to the outpatient department with easy fatigability, breathlessness, and pedal edema since 15 days with difficulty in swallowing solids for the past 1-year. On examination, she had pallor [Figure 1], angular stomatitis, bald tongue with atrophic papillae and koilonychia [Figure 2].
Investigations revealed microcytic hypochromic anemia with hemoglobin of 3.6 g%. Serum iron studies showed deceased serum iron of 25 μg/dl and increased total iron binding capacity of 498 μg/dl suggesting iron deficiency anemia. Upper GI endoscopy was done to find out the cause of dysphagia, which showed postcricoid web.
This patient had a triad of dysphagia, iron deficiency anemia, and upper esophageal webs. Therefore, a diagnosis of PVS was made. During endoscopy esophageal dilatation was done in view of symptomatic dysphagia. The patient was started on Iron supplementation and follow-up examination showed normal hemogram.
A 48-year-old male presented with a history of dysphagia, generalized weakness, and weight loss since 2 months. On physical examination, he had pallor, angular stomatits, and koilonychia [Figure 3]. All vitals were within normal limits.
Hematological tests were suggestive of iron deficiency anemia with hemoglobin of 6.2 g/dl, serum iron of 30 μg/dl, and serum ferritin of 4.8 ng/ml. 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. The patient was given iron replacement therapy for 6 months, and hematological tests normalized.
A 50-year-old female presented with a history of generalized weakness, weight loss, and dysphagia since 1-year. On examination, she had pallor [Figure 5], bald tongue, and koilonychia [Figure 6]. Laboratory investigations revealed hemoglobin of 6 g/dl, mean corpuscular volume of 56 fl, serum iron of 25 μg/dl, total iron binding capacity of 490 μg/dl, and serum ferritin of 5 ng/dl. Peripheral blood smear showed microcytic hypochromic anemia. Upper GI endoscopy showed postcricoid web.
Endoscopic dilatation of the web was done to relieve dysphagia. She was started on Iron supplementation. The patient was followed up, and there was improvement in the hematological parameters.
| Discussion|| |
PVS is one of the manifestations of long standing iron deficiency anemia. Most of the patients affected are middle-aged women at the age of 40–70 years. The dysphagia is progressive, limited to solids. It is also associated with significant weight loss. Anemic symptoms such as fatigue, breathlessness, and palpitation are the most common presenting symptom. It is also characterized by glossitis, angular stomatitis, and koilonychia. Rarely, splenomegaly and thyroid involvement can be seen.
The etiopathogenesis of PVS is not clear. Iron deficiency anemia is the most widely accepted causal association of PVS, and its role in the formation of postcricoid web has been studied. The etiopathogenesis of anemia is due to increased physiological demand, pathological demand, pathological blood loss, and inadequate iron intake. The iron deficiency anemia results in the loss of iron-dependent enzymes within the mucosal epithelial cells and high cell turnover. These results in mucosal degeneration, atrophic changes, web formation and can lead to cancer of the upper GI tract. The esophageal web is localized below the cricopharyngeal muscle and is asymmetrically attached to the anterior esophageal wall. Furthermore, iron deficiency results in the reduction of the iron-dependent oxidative enzymes, which results in the degradation of muscles of the pharynx. Iron deficiency also reduces the amplitude of contraction of the esophageal muscle resulting in motility impairment.
This syndrome has to be differentiated from other causes of dysphagia, malignancy, strictures, esophageal burns, achalasia, esophageal motility disorders, etc. The approach begins with laboratory evaluation, complete hemogram, with iron studies to establish iron deficiency anemia. Endoscopy may be used to find out the cause for dysphagia. Endoscopy also helps in obtaining biopsy samples to rule out other disorders with similar clinical presentation. All three patients had significant dysphagia and required esophageal dilatation using Savary–Gilliard dilator. In most of the cases, only one session of dilatation was sufficient.
PVS is considered a precancerous condition and is a risk factor for the development of squamous cell carcinoma. This disease is more common in females. In our case series, one of the patients was a male, and PVS in male is an uncommon presentation. All three patients are doing well after iron supplementation and esophageal dilatation.
| Conclusion|| |
There was a decline in the incidence of PVS because of improved nutritional status. However, in developing countries like India, where under nutrition is still a problem, PVS must be looked into in all patients presenting with anemia and dysphagia.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Novacek G. Plummer-Vinson syndrome. Orphanet J Rare Dis 2006;1:36.
Wynder EL, Hultberg S, Jacobsson F, Bross IJ. Environmental factors in cancer of the upper alimentary tract; a Swedish study with special reference to Plummer-Vinson (Paterson-Kelly) syndrome. Cancer 1957;10:470-87.
Hoffman RM, Jaffe PE. Plummer-Vinson syndrome. A case report and literature review. Arch Intern Med 1995;155:2008-11.
Anderson SR, Sinacori JT. Plummer-Vinson syndrome heralded by postcricoid carcinoma. Am J Otolaryngol 2007;28:22-4.
Standards of Practice Committee, Egan JV, Baron TH, Adler DG, Davila R, Faigel DO, et al
. Esophageal dilation. Gastrointest Endosc 2006;63:755-60.
Chisholm M. The association between webs, iron and post-cricoid carcinoma. Postgrad Med J 1974;50:215-9.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]