Plummer-Vinson syndrome differential diagnosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Akshun Kalia M.B.B.S.[2]

Overview

Plummer-Vinson syndrome must be differentiated from other diseases that cause dysphagia such as reflux esophagitis, esophageal carcinoma, systemic sclerosis, esophageal spasm, pseudoachalasia, stroke, esophageal candidiasis and Chagas disease.

Differential Diagnosis

Dysphagia

Oropharyngeal dysphagia

Esophageal dysphagia

Solids only

Solids and Liquids

Solids only

Solids and Liquids

•Zenker's diverticulum •Neoplasm •Webs

Neurogenic

Myogenic

Pain

•Achalasia •Scleroderma •DES

•Myasthenia gravis •Connective tissue disorder •Myotonic dystrophy

No

Yes

Heart burn

Barium swallow

Mental status

•Pill esophagitis •Caustic injury •Chemotherapy

Yes

No

Impaired

Normal

Non progressive

Progressive

Sac

Webs

Mass

Scleroderma

•Achalasia •DES

Stroke

•ALS •Parkinsonism

•Rings •Webs

•Strictures •Cancer

Zenker's diverticulum

Plummer-Vinson syndrome

Carcinoma

Chest pain and manometry

Barium swallow

Weight loss

Increase LES pressure

Rings

Webs

Yes

No

Rapid

Slow

Achalasia

DES

Cancer

Strictures/GERD

Plummer-Vinson syndrome must be differentiated from other diseases that cause dysphagia such as reflux esophagitis, esophageal carcinoma, systemic sclerosis, esophageal spasm, pseudoachalasia, stroke, esophageal candidiasis and Chagas disease.

Disease	Signs and Symptoms								Barium esophagogram	Endoscopy	Other imaging and laboratory findings	Gold Standard
	Onset	Dysphagia			Weight loss	Heartburn	Other findings	Mental status
		Solids	Liquids	Type
Plummer-Vinson syndrome	Gradual	+	-	Non progressive	+/-	-	Glossitis Koilonychia	Normal	Thin projections on the anterior esophageal wall Multiple upperesophageal constrictions	Direct visualization of esophageal webs Superior to esophagogram	Videofluoroscopy shows mucosal and submucosal foldings	Triad of Iron deficiency anemia Esophageal webs Glossitis
Esophageal stricture	Gradual or sudden	+	-	Progressive	+/-	+/-	Odynophagia Cough Chest pain	Normal	Sacculations Fixed transverse folds Esophageal intramural pseudodiverticula	Mucosal edema Circumferential thickening in GERD Pale mucosa with white exudate in lymphocytic esophagitis Swelling and hemorrhagic congestion in causticingestion	Manometry may show dysmotility CT scan for staging malignant strictures	Barium esophagogram
Diffuse esophageal spasm	Sudden	+	+	Non progressive	+	+	Chest pain	Normal	Nonperistaltic and nonpropulsive contractions Corkscrew or rosary bead esophagus	Inconclusive	Manometry shows high-amplitude esophageal contractions CT scan may show hypertrophy of esophageal muscles	Manometry
Achalasia	Gradual	+	+	Non progressive	+/-	-	Regurgitation of undigested food Chest pain	Normal	"Bird's beak" or "rat tail" appearance Dilated esophageal body Air fluid level (absent peristalsis) Absence of an intragastric air bubble	Dilated esophagus Residual food fragments Normal mucosa	Residual pressure of LES > 10 mmHg Incomplete relaxation of the LES Increased resting tone of LES Aperistalsis	History of dysphagia with positive endoscopy and manometry
Systemic sclerosis	Gradual	+	+	Progressive	+/-	+	Muscle and joint pain Raynaud's phenomenon Skin changes	Normal	Dysmotility Patulous esophagus	Mucosal damage Peptic stricture (advanced cases)	Positive serology for Antinuclear antibodies Rheumatoid factor Creatine kinase ESR	Skin biopsy
Zenker's diverticulum	Gradual	+	-		+/-	-	Food regurgitation Halitosis Cough Hoarseness	Normal	Thin projections on esophageal wall over Killian's triangle	Outpouching of posterior pharyngeal wall Exclude the presence of SCC	CT & MRI shows out-pouching over the posterior esophagus in the Killian's triangle	Barium esophagography
Esophageal carcinoma	Gradual	+	+	Progressive	+	+/-	Lymphadenopathy Cachexia	Normal	Irregular stricture Pre-stricture dilatation	Esophageal obstruction Staging of disease	CT and PET scan is an optional test for staging of the disease	Biopsy
Stroke (Cerebral hemorrhage)	Sudden	+	+	Progressive	+	+/-	Dysarthria Limb weakness Fatigue	Impaired	Pooling of contrast in the pharynx Aspiration of barium contrast into the airway	Reduced opening of upper esophageal sphincter Reduced larynx elevation	CT without contrast shows acute hemorrhage as a hyperattenuating clot	CT without contrast
Motor disorders (Myasthenia gravis)	Gradual	+	+	Progressive	+/-		Ptosis Diplopia Fatigue	Normal	Stasis in pharynx and pooling in pharyngeal recesses	Velopharyngeal insufficiency Delayed swallowing function	CT may show anterior mediastinal mass (thymoma) Positive tensilon test	Anti–acetylcholine receptor antibody test
GERD	Gradual or sudden onset	+	-	Progressive	+/-	+	Cough Hoarseness	Normal	Free acid reflux Esophagitis with scarring Strictures Barrett's oesophagus	Erythema, erosions and ulceration Barrett's esophagus	Esophageal manometry may show decreased tone of LES	24 hour esophageal pH monitoring
Esophageal web	Gradual	+	+/-	Progressive	-	+/-	Findings of the underlying cause such as iron deficiency anemia or bullous pemphigoid	Normal	Symmetrical narrowing of the esophagus	Smooth membrane not encircling the whole lumen	Videofluoroscopy shows mucosal and submucosal foldings	Barium esophagogram

Disease	Signs & Symptoms	Findings on barium esophagogram	Findings on endoscopy	Other findings
Reflux esophagitis	Dysphagia (from peptic stricture) Heartburn Hoarseness	Poor clearance Free reflux of barium	Peptic stricture (advanced cases)	Lower esophageal pH studies will demonstrate pathologic gastroesophageal reflux A hiatus hernia may be present below the stricture Manometry shows decreased tone of lower esophageal sphincter
Esophageal carcinoma	Dysphagia (initially for solids, liquids develops with advanced disease.) Weight loss Lymphadenopathy Cachexia	Irregular stricture Pre-stricture dilatation	Endoscopy with biopsy is the most accurate test for diagnosis and tumor histology. It may be used to depict: Esophageal obstruction Staging of disease	CT scan and PET scan of the chest and abdomen is an optional test for staging of the disease
Systemic sclerosis	Dysphagia Muscle and joint pain Raynaud's phenomenon Skin changes (rash, skin thickening)	Dysmotility Patulous esophagus	Mucosal damage Peptic stricture (advanced cases)	Positive serology for Antinuclear antibodies Rheumatoid factor Creatine kinase ESR
Esophageal spasm	Chest pain (more prominent) Dysphagia (intermittent)	Nonperistaltic and nonpropulsive contractions Corkscrew or rosary bead esophagus	Inconclusive	Manometry shows high-amplitude esophageal contractions CT scan may show show hypertrophy of esophageal muscle wall
Pseudoachalasia	Dysphagia Weight loss Lymphadenopathy Appetite changes Cachexia Older patients Underlying malignancy that mimics idiopathic achalasia. Patients tend to be older, duration of symptoms shorter, and weight loss greater and more rapid.	More marked mucosal irregularity Temporary patency of LES	Endoscopy with biopsy is the most accurate test for diagnosis and tumor histology. It may be used to depict: Esophageal obstruction Staging of disease	Gastroscopic biopsy of gastroesophageal junction and cardia may demonstrate malignancy. Findings at endoscopy, barium swallow, and manometry may be indistinguishable from achalasia.
Chagas disease	Dysphagia Toxic megacolon Myocarditis Blepharitis	Esophageal dilatation Stasis of barium	Dilated esophagus Thickened LES (muscular ring)	Giemsa stain will show Trypanosoma cruzi. PCR may be done to determine trypanosome subtype
Pharyngitis	Dysphagia Fever Throat pain	Normal	Inconclusive	Rapid antigen detection test positive for group A streptococccus Tonsillar hypertrophy may cause severe narrowing of the pharynx Physical exam may show: Erythema, edema and/or exudates of the pharynx Lymphadenopathy
Esophageal candidiasis	Dysphagia Immunocompromised History of corticosteroid	Shaggy" appearance (plaques) Irregular contours in the lower third	Ulceration  Plaques and pseudomembranes Tiny nodules, polypoid folds (advanced cases)	Creamy white or yellowish plaques (thrush) in oropharynx
Stroke	Progressive dysphagia Dysarthria Limb weakness Fatigue	Pooling of contrast in the pharynx Aspiration of barium contrast into the airway.	Reduced opening of upper esophageal sphincter Reduced larynx elevation	CT without contrast is the best initial test to differentiate between ischemic and hemorrhagic stroke MRI is more specific and sensitive than a CT scan but is more time consuming.

Plummer-Vinson syndrome must be differentiated from other causes of dysphagia, odynophagia and food regurgitation such as GERD, esophageal adenocarcinoma and esophageal stricture.

	Manifestations	Diagnostic tools
Achalasia	Dyspnea Dysphagia for solids and liquids is the most common feature, being seen in 91 % and 85% of patients respectively Regurgitation of undigested food occurs in 76-91% of patients Cough mainly when lying down in 30%	Esophagogastroduodenoscopy findings include a dilated esophagus with residual food fragments, normal mucosa and occasionally candidiasis (due to the prolonged stasis). Barium swallow shows the characteristic bird's beak appearance. Barium swallow showing bird's beak appearance - By Farnoosh Farrokhi, Michael F. Vaezi. - Idiopathic (primary) achalasia. Orphanet Journal of Rare Diseases 2007, 2:38(http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=2040141), CC BY 2.0, https://commons.wikimedia.org/w/index.php?curid=2950922
GERD	Retrosternal burning chest pain. Cough and hoarseness of voice. May present with complications such as strictures and dysphagia.	Upper GI endoscopy shows the complications such as esophagitis and barret esophagus. Esophageal manometry may show decreased tone of the lower esophageal sphincter. 24-hour esophageal pH monitoring may be done to confirm the diagnosis. Barret's esophagus - By Samir धर्म - taken from patient with permission to place in public domain, Copyrighted free use, https://commons.wikimedia.org/w/index.php?curid=1595945
Esophageal carcinoma	Dysphagia Odynophagia- fluids and soft foods are usually tolerated, while hard or bulky substances (such as bread or meat) cause much more difficulty Weight loss Pain, often of a burning nature, may be severe and worsened by swallowing, and can be spasmodic in character Nausea and vomiting	Upper GI endoscopy and esophageal biopsy the gold standard for the diagnosis of esophageal CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=2587715
Corckscrew esophagus	Retrosternal chest pain that presents with or without food intake. The condition is not progressive and not causing complications.	Barium swallow shows the characteristic corckscrew appearance of the esophagus. Corckscrew esophagus - Case courtesy of Radswiki, Radiopaedia.org, rID: 11680
Esophageal stricture	Patient may present with the symptoms of the underlying GERD. Dysphagia and odynophagia.	Barium esophagography provides information about the site and the diameter of the stricture before the endoscopic intervention. Peptic stricture - By Samir धर्म - From en.wikipedia.org, Public Domain, https://commons.wikimedia.org/w/index.php?curid=1931423
Plummer-Vinson syndrome	Common symptoms of Plummer-Vinson syndrome include: Difficulty swallowing (more for solids) Weakness Pain Burning sensation in mouth Dry tongue Painful cracks in the angles of a dry mouth Pale color of the skin Less common symptoms Cold intolerance Reduced resistance to infection Altered behavior Craving for for unusual items (such as ice or cold vegetables)	Lab tests are consistent with the diagnosis of iron deficiency anemia. Findings on an x-ray (barium esophagogram) suggestive of esophageal web/strictures associated with Plummer-Vinson syndrome appear as either: Thin projections on the anterior esophageal wall. Multiple upper (cervical) esophageal constrictions consistent with esophageal webs. Plummer-Vinson syndrome (Source: Case courtesy of Dr Hani Salam, <a href="https://radiopaedia.org/">Radiopaedia.org</a>. From the case <a href="https://radiopaedia.org/cases/14029">rID: 14029</a>)