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Table of Contents
CASE REPORT
Year : 2020  |  Volume : 8  |  Issue : 3  |  Page : 142-144

Plummer–Vinson syndrome: A case series


Department of General Medicine, Bangalore Medical College and Research Institute, Bengaluru, Karnataka, India

Date of Submission10-Sep-2019
Date of Decision15-Feb-2020
Date of Acceptance13-Mar-2020
Date of Web Publication15-Jul-2020

Correspondence Address:
Dr. Devapriya Rejeev
Devatharu, Kuniyedath House, Marikkunnu P O, Calicut - 673 012, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/AJIM.AJIM_51_19

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  Abstract 


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 Top


Paterson–Brown–Kelly syndrome or Plummer–Vinson syndrome (PVS) was reported by Henry Stanley Plummer in 1912 and Porter Paisley Vinson in 1919.[1] 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 Top


Case 1

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].
Figure 1: Case 1: Pallor with bald tongue

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Figure 2: Case 1: Koilonychia

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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.

Case 2

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.
Figure 3: Case 2: Male patient with koilonychia

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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].
Figure 4: Case 2: Esophageal webs on upper gastrointestinal endoscopy

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The postcricoid web was dilated using Savary–Gilliard dilator. The patient was given iron replacement therapy for 6 months, and hematological tests normalized.

Case 3

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.
Figure 5: Case 3: Pallor with bald tongue

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Figure 6: Case 3: Koilonychia

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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 Top


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.[2] 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.[3] 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.[4] 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.[5]

PVS is considered a precancerous condition and is a risk factor for the development of squamous cell carcinoma.[6] 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 Top


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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Novacek G. Plummer-Vinson syndrome. Orphanet J Rare Dis 2006;1:36.  Back to cited text no. 1
    
2.
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.  Back to cited text no. 2
    
3.
Hoffman RM, Jaffe PE. Plummer-Vinson syndrome. A case report and literature review. Arch Intern Med 1995;155:2008-11.  Back to cited text no. 3
    
4.
Anderson SR, Sinacori JT. Plummer-Vinson syndrome heralded by postcricoid carcinoma. Am J Otolaryngol 2007;28:22-4.  Back to cited text no. 4
    
5.
Standards of Practice Committee, Egan JV, Baron TH, Adler DG, Davila R, Faigel DO, et al. Esophageal dilation. Gastrointest Endosc 2006;63:755-60.  Back to cited text no. 5
    
6.
Chisholm M. The association between webs, iron and post-cricoid carcinoma. Postgrad Med J 1974;50:215-9.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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