Journal of Rare Disorders: Diagnosis & Therapy

Rapidly Progressive Plummer Vinson Syndrome

Context: Plummer Vinson Syndrome is diagnosed by a triad of dysphagia, esophageal webs, and iron deficiency anemia. These symptoms typically progress over a period of six months and often include other systemic effects such as angular cheilitis, glossitis, and spooning of the fingernails. Treatment includes iron supplementation as well as esophageal dilation. Annual upper endoscopic surveillance is an important measure for cancer surveillance.

Case report: A 47-year-old African American female with a history of hypertension presented to the emergency room with dysphagia. Over a period of two weeks, the patient first noticed difficulty swallowing large chunks of food followed shortly by trouble swallowing liquids. She described pain in her oropharynx with eating and regurgitated both solids and liquids every time she ate. She had lost 10 pounds over two weeks and described a new craving for ice (pica). Review of systems was otherwise negative. She denied chest pain, dyspnea, abdominal pain, hematemesis, and had no history of reflux. Physical exam was unremarkable. There was no evidence of glossitis, angular cheilitis, or spooning of the nail beds (koilonychia).

Conclusion: This case represents an atypical or rapid progression of Plummer Vinson Syndrome which developed over a period of approximately two weeks. Subsequently, the patient did not display the tell-tale systemic effects typically associated with this syndrome. Clinicians should be aware of similar presentations that fulfill the diagnostic criteria but circumvent the typical progression and associated sequelae. Indeed, recognizing this triad has important implications for esophageal cancer surveillance.

Author(s):

Michael Skolka and Helen Pauly-Hubbard

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