| Plummer–Vinson syndrome | |
|---|---|
| Classification and external resources | |
| ICD-10 | D50.1 |
| ICD-9 | 280.8 |
| DiseasesDB | 10134 |
| MedlinePlus | 001158 |
| eMedicine | med/3431 |
| MeSH | D011004 |
Plummer–Vinson syndrome (PVS), also called Paterson–Brown–Kelly syndrome or sideropenic dysphagia presents as a triad of dysphagia (due to esophageal webs), esophagitis, and iron deficiency anemia.[1] It most usually occurs in postmenopausal women.
Contents |
PVS sufferers often complain of a burning sensation with the tongue and oral mucosa, and atrophy of lingual papillae produces a smooth, shiny red tongue dorsum.
Symptoms include:
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.
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 over 50 years. 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, cheilitis, and splenomegaly.
The disease is named after two Americans, the physician Henry Stanley Plummer, and the surgeon Porter Paisley Vinson.[3][4][5]
It is also rarely called Kelly-Paterson syndrome, after Adam Brown-Kelly and Donald Ross Paterson.[3][6][7]
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]
Patients generally respond well to treatment. Iron supplementation usually resolves the anemia, and corrects the glossodynia (tongue pain).
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.
Good nutrition with adequate intake of iron may prevent this disorder.
|
||||||||||||||||||||||||||||||||||||||||||||||||||||||||||