Plummer Vinson syndrome

Other Names: Kelly's syndrome; Paterson-Kelly syndrome; Paterson-Brown-Kelly syndrome; Dysphagia sideropenica; Paterson’s syndrome; Kelly-Paterson syndrome; Plummer-Vinson syndrome; Sideropenic dysphagia

Plummer-Vinson or Paterson-Kelly syndrome presents as a classical triad of dysphagia, iron-deficiency anemia and esophageal webs. People with this condition have problems swallowing due to small, thin growths of tissue that partially block the upper food pipe (esophagus).

Epidemiology

Exact data about the epidemiology of the syndrome are not available; the syndrome is extremely rare.

Cause

The cause of Plummer-Vinson syndrome is unknown. The most important possible etiological factor is iron deficiency. Other possible factors include malnutrition, genetic predisposition or autoimmune processes. It is a rare disorder that can be linked to cancers of the esophagus and throat. It is more common in women.

Signs and symptoms

Most of the patients are white middle-aged women, in the fourth to seventh decade of life, but the syndrome has also been described in children and adolescents. The dysphagia is usually painless and intermittent or progressive over years, limited to solids and sometimes associated with weight loss. Symptoms resulting from anemia (weakness, pallor, fatigue, tachycardia) may dominate the clinical picture. Additional features are glossitis, angular cheilitis and koilonychia. Enlargement of the spleen and thyroid may also be observed. One of the most important clinical aspects of Plummer-Vinson syndrome is the association with upper alimentary tract cancers.

For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. 100% of people have these symptoms

• Decreased serum ferritin(Low ferritin level)
• Dysphagia(Poor swallowing)
• Esophageal web
• Hypochromic microcytic anemia
• Iron deficiency anemia

80%-99% of people have these symptoms

• Easy fatigability
• Glossitis(Inflammation of the tongue)
• Pallor

5%-29% of people have these symptoms

• Abdominal pain(Pain in stomach)
• Cheilitis(Inflammation of the lips)
• Concave nail(Spoon-shaped nails)
• Geophagia
• Intra-oral hyperpigmentation
• Narrow mouth(Small mouth)
• Poor appetite(Decreased appetite)
• Tongue atrophy(Wasting of the tongue)

Diagnosis

Your health care provider will do an exam to look for abnormal areas on your skin and nails. You may have an upper GI series or upper endoscopy to look for abnormal tissue in the food pipe. You may have tests to look for anemia or iron deficiency.

Treatment

Plummer-Vinson syndrome can be treated effectively with iron supplementation and mechanical dilation. In case of significant obstruction of the esophageal lumen by esophageal web and persistent dysphagia despite iron supplementation, rupture and dilation of the web are necessary. Since Plummer-Vinson syndrome is associated with an increased risk of squamous cell carcinoma of the pharynx and the esophagus, the patients should be followed closely.

Prognosis

People with this condition generally respond to treatment. Possible Complications Devices used to stretch the esophagus (dilators) may cause a tear. This can lead to bleeding.

Plummer-Vinson syndrome has been linked to esophageal cancer.

NIH genetic and rare disease info

• NIH info on Plummer Vinson syndrome

Plummer Vinson syndrome is a rare disease.

Plummer Vinson syndrome Resources
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