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, and esophageal webs.^[1] Treatment with iron supplementation and mechanical widening of the esophagus generally provides an excellent outcome.

Nowadays, this syndrome has become extremely rare.^[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]

Presentation

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:

• Dysphagia (difficulty in swallowing)
• Pain
• Weakness
• Odynophagia (painful swallowing)
• Atrophic glossitis
• Angular stomatitis

Serial contrasted gastrointestinal radiography or upper gastrointestinal endoscopy may reveal the web in the esophagus. Blood tests show 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.

Causes and associated conditions

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 (peak age is over 50). In these patients, esophageal squamous cell carcinoma risk is increased;^[1] therefore, it is considered a premalignant process.

The condition is associated with koilonychia, glossitis, inflammation of the lips (cheilitis), and splenomegaly.

Eponym

The disease is named after two Americans: the physician Henry Stanley Plummer and the surgeon Porter Paisley Vinson.^[3][4][5]

It is preferentially known as Kelly-Paterson syndrome in the UK, after Derek Brown-Kelly and Donald Ross Paterson.^[3][6][7]

Treatment

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.

If not, the web can be dilated during upper endoscopy to allow normal swallowing and passage of food.^[8]

Prognosis

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

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.^[9]

Prevention

Good nutrition with adequate intake of iron may prevent this disorder.

See also

• List of hematologic conditions

References

External links

• 00325 at CHORUS
• PVS Resource