Plummer-Vinson syndrome

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Overview

Plummer-Vinson syndrome (also called Patterson-Kelly syndrome or sideropenic dysphagia) is a rare condition that combines three main problems: difficulty swallowing, iron-deficiency anemia, and thin web-like growths of tissue in the upper part of the esophagus (the tube that carries food from your mouth to your stomach). These webs can partially block the esophagus, making it hard to swallow solid foods. The anemia means your blood is not carrying enough oxygen, which causes tiredness, weakness, and pale skin. The condition most often affects middle-aged women, though it can occur in anyone. Doctors believe it develops because of long-term, severe iron deficiency, though the exact cause is not fully understood. Other symptoms can include a sore, smooth tongue, cracked corners of the mouth, brittle nails, and an enlarged spleen in some cases. The good news is that Plummer-Vinson syndrome usually responds well to treatment. Iron supplementation can correct the anemia and may help the esophageal webs improve on their own. If the webs are causing significant swallowing problems, a doctor can gently stretch or break them open during an endoscopy procedure. With proper treatment, most people do very well. However, it is important to know that this condition has been linked to a higher risk of developing cancer in the throat or esophagus over time, so regular monitoring is recommended.

Also known as:

Key symptoms:

Difficulty swallowing solid foodsExtreme tiredness and low energy due to anemiaPale skinWeakness and dizzinessSore, smooth, or burning tongue (glossitis)Cracked or sore corners of the mouth (angular cheilitis)Brittle or spoon-shaped nails (koilonychia)Shortness of breathRapid or irregular heartbeatUnintended weight loss due to difficulty eatingFeeling of food getting stuck in the throat or upper chestEnlarged spleen (in some cases)Hair loss or thinning

Clinical phenotype terms (16)— hover any for plain English
Hypochromic microcytic anemiaHP:0004840Decreased circulating ferritin concentrationHP:0012343Esophageal webHP:0100594GlossitisHP:0000206Easy fatigabilityHP:0003388Concave nailHP:0001598Intra-oral hyperpigmentationHP:0010284Tongue atrophyHP:0012473GeophagiaHP:0025062CheilitisHP:0100825
Inheritance

Multifactorial

Caused by a mix of several genes and environmental factors

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Plummer-Vinson syndrome.

View clinical trials →

No actively recruiting trials found for Plummer-Vinson syndrome at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Plummer-Vinson syndrome community →

No specialists are currently listed for Plummer-Vinson syndrome.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Plummer-Vinson syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Plummer-Vinson syndrome

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Caregiver Resources

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Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.How long will I need to take iron supplements, and how will we know when my iron levels are back to normal?,Do my esophageal webs need to be treated with dilation, or can they improve with iron treatment alone?,What is my personal risk of developing throat or esophageal cancer, and how often should I be monitored?,Are there dietary changes I should make to help increase my iron levels naturally?,What symptoms should prompt me to seek urgent medical care?,Should I see any other specialists, such as a dietitian or an ENT doctor?,What is the expected timeline for my swallowing to improve with treatment?

Common questions about Plummer-Vinson syndrome

What is Plummer-Vinson syndrome?

Plummer-Vinson syndrome (also called Patterson-Kelly syndrome or sideropenic dysphagia) is a rare condition that combines three main problems: difficulty swallowing, iron-deficiency anemia, and thin web-like growths of tissue in the upper part of the esophagus (the tube that carries food from your mouth to your stomach). These webs can partially block the esophagus, making it hard to swallow solid foods. The anemia means your blood is not carrying enough oxygen, which causes tiredness, weakness, and pale skin. The condition most often affects middle-aged women, though it can occur in anyone.

How is Plummer-Vinson syndrome inherited?

Plummer-Vinson syndrome follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Plummer-Vinson syndrome typically begin?

Typical onset of Plummer-Vinson syndrome is adult. Age of onset can vary across affected individuals.