A middle aged woman presents with difficulty swallowing. She has a history of menometrorrhagia for 10 years, secondary to uterine fibroids. She endorses fatigue and shortness of breath.
What’s the cause of her dysphagia and why?
Esophageal webs in the setting of iron deficiency anemia. This is seen in Plummer-Vinson syndrome. This syndrome usually has:
- esophageal web
- atrophic glossitis (inflammation of tongue) and/or cheilitis (inflammation around the lips/mouth)
- iron deficiency anemia
This patient presents with koilonychia (spoon-shaped nail) and platynychia (flat nails) which should make you think of iron deficiency anemia. Given her dysphagia, esophageal webs are highest on the differential. A barium esophagram (figure below) will confirm the diagnosis. The esophageal webs of Plummer-Vinson syndrome are usually in the upper esophagus and more common in women. They are thought of as a pre-cancer as the risk of squamous cell carcinoma of the esophagus and pharynx is increased. If the lower esophagus is involved, it often presents as a lower esophageal stricture or ring (i.e. Schatzki ring).
What are other common findings in iron deficiency anemia?
- Any symptom of anemia (shortness of breath, fatigue, palpitations)
- Pallor – conjuctival pallor is the best place to look but if the anemia is severe, pallor can be generalized
- Koilonychia (spoon-shaped nail) and platynychia (flat nails) – as seen above
- Pica – an atypical appetite for uncommon things (dirt, clay, paper). Pagophagia or pica for ice is often very specific for iron deficieny and can be seen before anemia occurs when iron stores are low.
- Restless legs syndrome (RLS) – iron deficiency is one of the more common causes of RLS
- Beeturia – due to an increased absorption of the red pigment of beats called betalaine in iron deficiency anemia, patients with iron deficiency anemia are more likely to excrete this reddish pigment and have red urine. (Note for patients without iron deficiency anemia it still possible to absorb and excrete this pigment, it’s just much less common.)
What are causes of iron deficiency anemia?
- Blood loss
- Severe menorrhagia
- Gastrointestinal blood loss
- Traumatic hemorrhage
- Pulmonary hemorrhage
- Decreased iron absorption
- Celiac disease
- Medications – less commonly, medications such as proton pump inhibitors can affect iron absorption
- Other causes
- Erythropoietin – causing increase production of RBC’s can increase demand for iron depleting stores
- Intravascular hemolysis – lysis of RBC’s in blood followed by loss in urine can deplete stores
- Gastric bypass – duodenum is main site of iron absorption
- Congenital causes
Why esophageal webs in Plummer-Vinson Syndrome?
The actual mechanism is unknown but since iron acts as a co-factor to many enzymes, it is thought that the lack of iron to act as co-factors to enzymes specific to the esophagus affects epithelial turnover and leads to the webs.
What’s where the does name Plummer-Vinson Syndrome come from?
This name comes from two Americans named Henry Stanley Plummer and Porter Paisley Vinson. Dr. Plummer was a prominent internist and endocrinologist born in 1874 and was one of the founding members of the Mayo Clinic. Dr. Vinson was a surgeon born in 1890 and also worked at the Mayo Clinic. The alternative name of Kelly-Paterson syndrome has been used in the UK after doctors Derek Brown-Kelly and Donald Ross Paterson.
What happened to the patient?
The patient was started on IV and later oral iron. After 4 months, an EGD showed resolution of the esophageal webs and her symptoms of dysphagia. She was also started on a depot of luprorelin to minimize her blood loss during menstruation until menopause.
- This case was obtained from Clinical Case Reports article Spooning of the nails and webbing of the esophagus: koilonychia and Plummer–Vinson Syndrome
- Samad, Mohan, Balaji , Augustine, Patil SG. Oral manifestations of plummer-vinson syndrome: a classic report with literature review. J Int Oral Health. 2015 Mar;7(3):68-71.
- Angular cheilitis image: https://commons.wikimedia.org/wiki/File:Angular_cheilitis1.jpg
- Glossitis image: https://commons.wikimedia.org/wiki/File:Glossitis.jpg