Medical Mystery of the Week

An 82-year-old woman presents with a chief complaint of progressive difficulty in swallowing solid foods. You are provided with a picture of her thumb (see below) and asked to make a diagnosis. What is your diagnosis, what test(s) would you order to confirm your suspicions, and what treatment would you recommend?

thumb picture  esophogram

DIAGNOSIS: Plummer-Vinson syndrome. Also called the Paterson-Kelly or Patterson-Brown Kelly syndrome, this disorder presents with symptoms and signs of a long-standing iron deficiency (weakness, fatigue, angular cheilitis, glossitis, spoon nails [koilonychia], and occasional splenomegaly) and dysphagia for solids due to esophageal webbing. Most patients with this syndrome are caucasian women in their fourth to seventh decade of life. The dysphagia is usually painless and intermittent or progressive over a number of years. Three to 15% of patients with the syndrome develop squamous cell carcinoma of the pharynx or esophagus; thus, patients with this diagnosis must be monitored closely for this complication.

The presence of iron deficiency is confirmed by demonstrating low serum levels of iron and ferritin; the associated anemia will be hypochromic and microcytic. A careful search for the cause of the iron deficiency (dietary deficiency, occult bleeding, etc.) should be undertaken. An esophagram using barium or a water soluble contrast material will generally suffice to demonstrate the presence of webbing in the upper (cervical) esophagus (see above). 

Patients are treated with iron supplementation and, if needed, esophageal dilation.

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