WBR0774

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Author [[PageAuthor::Yazan Daaboul, M.D. (Reviewed by Serge Korjian)]]
Exam Type ExamType::USMLE Step 1
Main Category MainCategory::Pathology
Sub Category SubCategory::Gastrointestinal
Prompt [[Prompt::A 48-year-old woman with no past medical history presents to the physician's office with complaints of difficulty swallowing solid food for the past year. She explains that her condition has recently got worse, but denies difficulty swallowing liquids or odynophagia. Review of systems reveals significant fatigue and an unintentional weight loss of 4kg (9 lbs) in the past few months. Her blood pressure is 132/68 mm Hg, heart rate is 103/min, and temperature is 36.7 ᵒC (98 ᵒF). Physical examination is remarkable for skin pallor, fissuring of both corners of the mouth, and an inflammed and depapillated anterior tongue. The physician decides to perform an upper endoscopy for further evaluation. What is the most likely finding on endoscopy associated with this patient's condition?]]
Answer A AnswerA::Zenker's diverticulum
Answer A Explanation AnswerAExp::Zenker's diverticulum is not associated with Plummer-Vinson syndrome. It is caused by impaired relaxation of the cricopharyngeus muscle.
Answer B AnswerB::Esophageal webs
Answer B Explanation AnswerBExp::Esophageal webs are associated with Plummer-Vinson syndrome.
Answer C AnswerC::Esophageal strictures
Answer C Explanation AnswerCExp::Esophageal strictures are not associated with Plummer-Vinson syndrome.
Answer D AnswerD::Achalasia of the esophagus
Answer D Explanation AnswerDExp::Achalasia is associated with Chagas disease. It is not associated with Plummer-Vinson syndrome.
Answer E AnswerE::Esophageal esophagitis
Answer E Explanation AnswerEExp::Esophageal esophagitis is not associated with Plummer-Vinson syndrome.
Right Answer RightAnswer::B
Explanation [[Explanation::Plummer-Vinson syndrome (also known as Paterson-Kelly syndrome) is a rare disorder characterized by a triad of: dysphagia due to esophageal webs, glossitis (inflammation of the tongue), and iron deficiency anemia (IDA). Patients are classically middle-aged women, but age range is very wide as the disease has been described in children and elderly patients. Patients typically present with dysphagia to solids (not associated with odynophagia) that progresses over several years. Other signs and symptoms are due to associated conditions, such as skin pallor, fatigue, and tachycardia due to iron deficiency anemia. Additional findings on physical examination may include angular cheilitis (inflammation of labial commissures), koilonychia (nail spooning), palpable spleen, and/or palpable thyroid. The real etiology of Plummer-Vinson syndrome has not yet been identified, but several environmental and genetic predispositions have been described, including malnutrition and autoimmune diseases. The syndrome can be treated by iron replacement and mechanical dilation of the esophageal webs. Most importantly, Plummer-Vinson syndrome is associated with squamous cell carcinoma of the pharynx and the esophagus. Accordingly, patients diagnosed with this syndrome require close follow-up.

Educational Objective: Plummer-Vinson syndrome is a triad of glossitis, dysphagia due to esophageal webs, and iron deficiency anemia.
References: Novacek, G. Plummer-Vinson syndrome. Orphanet J Rare Dis. 2006; 1:36.]]

Approved Approved::Yes
Keyword WBRKeyword::Plummer-Vinson syndrome, WBRKeyword::Glossitis, WBRKeyword::Esophageal web, WBRKeyword::Esophagus, WBRKeyword::Koilonychia, WBRKeyword::Cheilitis, WBRKeyword::Dysphagia, WBRKeyword::Iron deficiency anemia, WBRKeyword::Esophageal squamous cell carcinoma, WBRKeyword::Squamous cell carcinoma, WBRKeyword::Plummer-Vinson
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