Plummer–Vinson syndrome

Plummer–Vinson syndrome

Plummer–Vinson syndrome (PVS), also called Paterson–Brown–Kelly syndrome or sideropenic dysphagia, is a rare disease characterized by difficulty in swallowing, iron-deficiency anemia, glossitis, cheilosis and esophageal webs.[1] 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 PVS has been hypothesized to be the result of improvements in nutritional status and availability in countries where the syndrome was previously described.[1] It generally occurs in postmenopausal women. Its identification and follow-up is considered relevant due to increased risk of squamous cell carcinomas of the esophagus and pharynx.[1]

Clinical presentation

Angular stomatitis

PVS sufferers often complain of 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:

Serial contrasted gastrointestinal radiography or upper-gastrointestinal endoscopy may reveal the web in the esophagus.

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. Epithelial atypia or dysplasia 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 PVS is unknown; however, genetic factors and nutritional deficiencies may play a role. It is more common in women,[2] particularly in middle age, with a peak age over 50 years. In these patients, esophageal squamous cell carcinoma risk is increased;[1] therefore, it is considered a premalignant process.[3]

The condition is associated with koilonychia, glossitis, inflammation of the lips (cheilitis), and splenomegaly. Esophageal web in Plummer–Vinson syndrome is found at upper end of esophagus (post cricoid region) and Schatzki ring may be found at the lower end of esophagus.[4]

Diagnosis

Following clinical presentations may be used in the diagnosis of this condition.

  1. Dizziness
  2. Pallor of the conjuctiva and face
  3. Erythematous oral mucosa with burning sensation
  4. Breathlessness
  5. Atrophic and smooth tongue
  6. Peripheral rhagades around the oral cavity

The following tests are helpful in the diagnosis of Plummer–Vinson syndrome.

Lab tests

complete blood cell (CBC) counts, peripheral blood smears, and iron studies (e.g., serum iron, total iron-binding capacity [TIBC], ferritin, saturation percentage) to confirm iron deficiency, 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.

Prevention

Good nutrition with adequate intake of iron may prevent this disorder. Good nutrition should also include balanced diet and exercise.

Treatment

Ascorbic-acid

Treatment is primarily aimed at correcting the iron-deficiency anemia. Patients with PVS should receive iron supplementation in their diet. This may improve dysphagia and pain.[1] If not, the web can be dilated during upper endoscopy to allow normal swallowing and passage of food.[5]

Prognosis

Patients generally respond well to treatment. Iron supplementation usually resolves the anemia, and corrects the glossodynia (tongue pain).[1]

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 carcinoma of the oral cavity, esophagus, and hypopharynx.[6]

History

The disease is named after two Americans: the physician Henry Stanley Plummer and the surgeon Porter Paisley Vinson.[7][8][9] It is occasionally known as Paterson-Kelly or Paterson-Brown Kelly syndrome in the UK, after Derek Brown-Kelly and Donald Ross Paterson.[10] however, Plummer–Vinson syndrome is still the most commonly used name.[10]

See also

References

  1. 1 2 3 4 5 6 Novacek G (2006). "Plummer-Vinson syndrome". Orphanet J Rare Dis. 1: 36. doi:10.1186/1750-1172-1-36. PMC 1586011. PMID 16978405.
  2. ↑ "Plummer-Vinson syndrome: MedlinePlus Medical Encyclopedia". 2011.
  3. ↑ Goel A, Bakshi SS, Soni N, Chhavi N. Iron deficiency anemia and Plummer-Vinson syndrome: current insights. J Blood Med. 2017 Oct 19;8:175-184. doi:10.2147/JBM.S127801
  4. ↑ Sabiston 18th/e p1078
  5. ↑ Enomoto M, Kohmoto M, Arafa UA, et al. (2007). "Plummer-Vinson syndrome successfully treated by endoscopic dilatation". J. Gastroenterol. Hepatol. 22 (12): 2348–51. doi:10.1111/j.1440-1746.2006.03430.x. PMID 18031398.
  6. ↑ "Plummer-Vinson Syndrome". PubMed Health. Retrieved 31 January 2012.
  7. ↑ synd/1777 at Who Named It?
  8. ↑ H. S. Plummer. Diffuse dilatation of the esophagus without anatomic stenosis (cardiospasm). A report of ninety-one cases. Journal of the American Medical Association, Chicago, 1912, 58: 2013-2015.
  9. ↑ 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.
  10. 1 2 Novacek, Gottfried (2006). Orphanet Journal of Rare Diseases. 1 (1): 36. doi:10.1186/1750-1172-1-36. Missing or empty |title= (help)
Classification
External resources
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