Plummer-Vinson or Paterson-
Kelly syndrome presents as a classical triad of
dysphagia,
iron-deficiency anemia and esophageal webs. Exact data about epidemiology of the syndrome are not available; the syndrome is extremely rare. Most of the patients are white middle-aged women, in the fourth to seventh decade of life but the syndrome has also been described in children and adolescents. The
dysphagia is usually painless and intermittent or progressive over years, limited to solids and sometimes associated with
weight loss. Symptoms resulting from
anemia (weakness, pallor,
fatigue,
tachycardia) may dominate the clinical picture. Additional features are
glossitis, angular
cheilitis and koilonychia. Enlargement of the spleen and thyroid may also be observed. One of the most important clinical aspects of
Plummer-Vinson syndrome is the association with upper alimentary tract
cancers. Etiopathogenesis of
Plummer-Vinson syndrome is unknown. The most important possible etiological factor is
iron deficiency. Other possible factors include
malnutrition,
genetic predisposition or autoimmune processes.
Plummer-Vinson syndrome can be treated effectively with
iron supplementation and mechanical dilation. In case of significant obstruction of the esophageal lumen by esophageal web and persistent
dysphagia despite
iron supplementation,
rupture and dilation of the web are necessary. Since
Plummer-Vinson syndrome is associated with an increased risk of
squamous cell carcinoma of the pharynx and the esophagus, the patients should be followed closely.