Plummer Vinson Syndrome in a Young African American Female: A Rare Entity

Abstract

Plummer Vinson syndrome (PVS), also known as Paterson-Brown-Kelly syndrome, is a rare clinical condition characterized by a triad of iron deficiency anemia, esophageal webs, and dysphagia. PVS has been rarely reported in African American females. In this case, however, we report a 26-year-old African American female with a 10-year history of iron deficiency anemia, who presented with severe fatigue and dysphagia. Her work-up revealed severe iron deficiency anemia, and upper endoscopy showed two esophageal rings. A 26-year-old African American female presented with severe fatigue, headache, and dysphagia for 5 months. The dysphagia was predominantly to solid foods compared to liquids. Patient also has a 10-year history of iron deficiency anemia and has stopped taking iron supplementations for the last 2 years. Physical examination revealed severe conjunctival pallor but was otherwise normal. Routine investigations revealed severe microcytic anemia with hemoglobin of 4.2 g/dL, mean corpuscular volume of 62.5 fL, and a ferritin level of 2 ng/dL. A modified barium swallow study showed two esophageal impressions in the cervical region, which were concerning for esophageal webs. Patient later underwent an esophagogastroduodenoscopy (EGD) which showed two upper esophageal rings; one semi-circumferential at 15 cm and a second circumferential at 17 cm from the incisors. A pediatric size scope was used to bypass the rings. She was initiated on iron replacement therapy with no symptomatic improvement of her dysphagia after 6 months of treatment. She then had a follow up EGD with wire-guided savary dilation with 8 mm dilator which resulted in an immediate improvement of her symptoms. Patient remained symptom free in her 2 months follow-up. PVS is characterized by iron deficiency anemia, esophageal webs, and dysphagia. It is predominantly seen in middle aged Caucasian females. Our case is unique because only a few cases of PVS have been reported in African American females. PVS is also a precancerous condition. Therefore, patients with this syndrome are at increased risk of developing gastrointestinal squamous cell carcinomas. Since nutritional deficiency is one of the major causes of iron deficiency anemia, the improvement in nutritional status of populations worldwide has led to a decreased incidence of PVS. Dysphagia in PVS usually responds to iron supplementation. However, resistant cases may require endoscopic dilatation of the webs as in our patient.1776_A Figure 1. EGD image Showing the first upper esophageal web, 15 cm from the incisors.1776_B Figure 2. EGD image Showing the second esophageal web, 17 cm from the incisors.1776_C Figure 3. Esophageal ring post wire-guided savary dilation with a satisfactory mucosal tear shown.