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Plummer-Vinson Syndrome - Causes, Symptoms, Diagnosis, and Treatment

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Plummer-Vinson syndrome is a disorder caused by long-lasting iron-deficiency anemia. Read this article to learn about it.

Medically reviewed by

Dr. Kaushal Bhavsar

Published At December 2, 2022
Reviewed AtApril 18, 2023

Introduction:

Plummer-Vinson syndrome (PVS) is a rare condition described by the triad of dysphagia (difficulty swallowing), iron-deficiency anemia, and esophageal webbing (thin esophageal tissue membranes consisting of mucosa obstructing the esophagus). This syndrome usually occurs after a long-lasting or chronic iron deficiency anemia. It is associated with an increased risk of developing squamous cell carcinoma of the pharynx and proximal esophagus. This article will discuss Plummer-Vinson syndrome in detail.

What Is Plummer-Vinson Syndrome?

Plummer-Vinson syndrome (PVS) is also known as Paterson-Brown-Kelly syndrome, derived from two physicians - Hh2enry Stanley Plummer and Porter Paisley Vinson. However, this syndrome is rare nowadays, but the diagnosis of this syndrome helps in the identification of patients at increased risk of squamous cell carcinoma of the pharynx and the esophagus.

What Is the Triad of Plummer-Vinson Syndrome?

The Plummer-Vinson syndrome triad includes dysphagia, iron-deficiency anemia, and esophageal webbing. However, the triad of PVS is not seen in all patients with esophageal webbing. Dysphagia and iron deficiency anemia are the most common occurring features.

  • Dysphagia - Dysphagia means difficulty swallowing and has a gradual or slow onset in PVS patients. Dysphagia is usually noticed in solid foods, followed by several years of difficulty in swallowing liquids, thus explaining the slow progression of esophageal obstruction. The patient usually points to the neck or above the suprasternal notch (a large, visible dip in between the neck in humans) as the site of obstruction. The patient can reduce the symptoms by altering the diet, and the obstruction may result in the person taking a long time to complete meals.

  • Iron Deficiency Anemia - Iron deficiency anemia is the most common type of anemia due to iron deficiency in the body, which our body needs to make hemoglobin. Anemia can present symptoms such as fatigue, exertional dyspnea, weakness, palpitations, etc. Along with all these symptoms, iron deficiency anemia can occur with deficiencies of other nutrients or vitamins, which can lead to other symptoms such as

    • Soreness of mouth.

    • Glossitis (inflammation of the tongue).

    • Angular cheilitis (a common skin condition affecting the corners of the mouth).

    • Atrophic glossitis (a condition in which many of the small bumps of the tongue or papillae shrink, which changes the surface of the tongue, making it appear glossy).

    • Premature loss of teeth.

    • Koilonychia (spoon-shaped fingernails).

    • Seborrheic dermatitis (a skin condition affecting the scalp).

    • Hyperkeratosis (a condition causing thickening of the outer layer of the skin, which is made of keratin).

    • Conjunctivitis (inflammation or infection of the eyeball and the inner eyelid).

    • Keratitis (inflammation of the clear tissue of the eye or cornea).

    • Paresthesia.

    • Night blindness.

Moreover, women may experience complaints of burning and itching in the vulval region, which usually disappears after the iron deficiency treatment.

  • Esophageal Webbing - Abnormal movement of muscles in the pharynx and esophagus leads to web formation. Iron deficiency anemia may also cause decreased esophageal motility due to impaired mitochondrial function and damaged DNA (Deoxyribonucleic acid). These damaged or impaired mitochondria have been linked to diseases like cancer, aging, and web formation. Esophageal webs are thin, circular, or semi-circular, pink-whitish, membranous structures consisting of two layers of mucosa. These are located mainly in the upper esophagus region, just below the esophageal sphincter. This location of the web makes it difficult to obtain a biopsy of a web.

What Is the Cause of Plummer-Vinson Syndrome?

The cause of Plummer-Vinson syndrome is unknown; however, various genetic factors or nutrient deficiencies may be the reason for its occurrence. It is more common in women, usually in their middle age, mainly after 50 years of age. This disorder can also be linked with cancer of the esophagus and throat.

What Are the Common Symptoms of Plummer-Vinson Syndrome?

Patients with Plummer-Vinson syndrome involve various symptoms related to iron deficiency anemia, such as a burning sensation on the tongue and oral mucosa and atrophy of tongue papillae giving a smooth, shiny, red surface of the tongue.

Along with all these symptoms, some other symptoms involve -

  • Dysphagia (difficulty swallowing).

  • Pain.

  • Weakness.

  • Odynophagia (painful swallowing).

  • Atrophic glossitis (a condition characterized by the absence of filiform or fungiform papillae on the dorsal surface of the tongue).

  • Angular cheilitis (skin condition causing inflammation and small cracks at the corners of the mouth).

  • Koilonychia (abnormally thin nails, also called spoon nails).

  • Splenomegaly (an enlarged spleen).

How to Diagnose Plummer-Vinson Syndrome?

Plummer-Vinson syndrome is a rare disorder mainly seen in patients with iron deficiency anemia, esophageal webs, and postcricoid dysphagia. Some various tests or investigations can be done to diagnose Plummer-Vinson syndrome, which involves -

  1. Blood Tests - Blood tests are done to check the cause of iron deficiency anemia and the severity of anemia.

  2. Radiographic Barium Swallow Tests - This test is the gold standard in diagnosing esophageal webs as they are widely available and can be easily recreated.

  3. Video-fluoroscopy - This is the most reliable diagnostic tool that can also be done for the diagnosis of esophageal webs. This technique uses a dynamic radiographic evaluation using x-rays where the barium-marked food can be seen passing from the oral cavity to the food pipe. These are beneficial as they help diagnose the smaller esophageal webs but also help in differentiating the true webs from the false ones.

  4. Fiberoptic Endoscopy - It is the safest and most reliable tool for gastrointestinal tract examination.

  5. Esophagoscopy - Endoscopic examination of the esophagus is a beneficial diagnostic and therapeutic tool. In this technique, the esophageal webs appear as smooth, thin, gray lesions and have normal mucosa, most commonly seen on the anterior wall of the esophagus.

  6. Biopsy - In some cases, a biopsy of the involved mucosa can be taken, which usually reveals epithelial atrophy and chronic inflammation.

What Is the Treatment or Management for Plummer-Vinson Syndrome?

The first step in treating Plummer-Vinson syndrome is to treat the cause of iron deficiency anemia to exclude active hemorrhage or malignancy. Iron supplements can be given to patients to treat dysphagia and iron deficiency anemia.

However, in cases with complete obstruction of the esophageal lumen by the esophageal web and dysphagia, a more invasive method should be followed to rupture and dilate the web. This can be done by performing endoscopy, which is inserting a tube or guidewire in the esophagus along with the dilators, which can be used to dilate up to 17 mm.

In most cases, one dilation is enough to treat the cause and relieve dysphagia, but sometimes multiple sessions can be required.

As Plummer-Vinson syndrome is associated with a high risk of squamous cell carcinoma of the pharynx and the esophagus, the patients should be followed closely. And patients should be advised to take a yearly endoscopy test to avoid complications.

What Is the Prognosis of Plummer-Vinson Syndrome?

Patients suffering from Plummer-Vinson syndrome have an excellent prognosis, with most patients requiring only one-time dilation of the esophagus or esophageal web for the complete relief of symptoms and iron replacement therapy. If associated with squamous cell carcinoma of the hypopharynx or upper esophagus, the prognosis worsens dramatically.

What Are the Complications of Plummer-Vinson Syndrome?

Some of the complications related to Plummer-Vinson syndrome are as follows -

  1. Esophageal webs can lead to dysphagia.

  2. Patients with iron deficiency anemia can develop symptomatic anemia if treatment is not done.

  3. In some cases, patients may develop squamous cell cancer of the proximal esophagus, worsening the condition.

  4. There is a rare chance of esophageal perforation by the endoscopic treatment of the esophageal web by balloon dilatations.

Conclusion:

Plummer-Vinsonsyndrome is a rare condition, and the knowledge of this condition is limited. Patients should always be aware of the symptoms related to this disease and should seek prompt medical advice. Importance should be given to patient awareness and education to prevent life-threatening consequences. Early diagnosis can prevent severe complications like squamous cell cancer.

Dr. Kaushal Bhavsar
Dr. Kaushal Bhavsar

Pulmonology (Asthma Doctors)

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