Plummer–Vinson syndrome

Angular Cheilitis 2

Plummer–Vinson syndrome (PVS), also known as Paterson-Brown-Kelly syndrome or sideropenic dysphagia, is a rare condition characterized by a triad of dysphagia, iron deficiency anemia, and esophageal webs. This syndrome predominantly affects middle-aged women and is associated with an increased risk of developing esophageal squamous cell carcinoma.

Signs and Symptoms

The primary symptoms of Plummer–Vinson syndrome include:

• Dysphagia (difficulty swallowing), often due to the presence of esophageal webs.
• Iron deficiency anemia, which can lead to symptoms such as fatigue, pallor, and weakness.
• Glossitis (inflammation of the tongue) and cheilosis (cracks at the corners of the mouth).

Other symptoms may include:

• Koilonychia (spoon-shaped nails)
• Splenomegaly (enlarged spleen)
• Atrophic gastritis

Pathophysiology

The exact cause of Plummer–Vinson syndrome is not well understood. However, it is believed that iron deficiency plays a crucial role in the development of esophageal webs and other mucosal changes. Iron is essential for the maintenance of epithelial integrity, and its deficiency may lead to mucosal atrophy and the formation of webs.

Diagnosis

Diagnosis of Plummer–Vinson syndrome is based on clinical presentation and confirmed through various diagnostic tests, including:

• Barium swallow radiography, which can reveal the presence of esophageal webs.
• Endoscopy, which allows direct visualization and potential biopsy of the esophageal webs.
• Blood tests to confirm iron deficiency anemia, including complete blood count (CBC) and serum iron studies.

Treatment

The primary treatment for Plummer–Vinson syndrome involves iron supplementation to correct the iron deficiency anemia. This can lead to the resolution of dysphagia and other symptoms. In some cases, mechanical dilation of the esophagus may be necessary to treat persistent esophageal webs.

Prognosis

With appropriate treatment, the prognosis for individuals with Plummer–Vinson syndrome is generally good. However, there is an increased risk of developing esophageal squamous cell carcinoma, necessitating regular monitoring and follow-up.

Related Pages

• Dysphagia
• Iron deficiency anemia
• Esophageal webs
• Esophageal squamous cell carcinoma
• Glossitis
• Cheilosis
• Koilonychia
• Atrophic gastritis