Spooning of the nails and webbing of the esophagus: koilonychia and P lummer– V inson S yndrome

Abstract

Key Clinical Message

Chronic iron deficiency can be associated with nail deformities like Koilonychia and Platynychia. It can also be associated with esophageal webs (Plummer‐Vinson syndrome or Patterson‐Brown‐Kelly syndrome) causing dysphagia in the patient. Though the pathogenesis of this association remains anecdotal and presence of these physical findings should prompt the clinician towards considering chronic iron deficiency as the cause of anemia.

A middle aged woman with history of four successful vaginal deliveries and with past medical history of menometrorrhagia for 10 years, secondary to fibroid uterus, presented with complaints of fatigue and shortness of breath. On a previous admission, in 2011, she had complained of dysphagia. At that time, she was found to have severe anemia with hemoglobin (Hb) of 6.6gm/dL, mean corpuscular volume (MCV) of 56.2 fL, and a ferritin of 0.8 ng/mL. She underwent esophago‐gastro‐duodenoscopy (EGD), which was suggestive of proximal esophageal web. Barium swallow study confirmed a 6 mm focal narrowing of the proximal esophagus (Fig. ). She was diagnosed with iron deficiency anemia and was treated with oral iron replacement therapy upon discharge. On the current admission (in the year 2013), vital signs were unremarkable and physical exam was significant for marked pallor, glossitis, platynychia, and koilonychias (Fig. ). Complete blood count showed white cell count of 4.6k/μL (4.4–10.6k/μL), Hb of 6.8gm/dL (11.7–14.9gm/dL), hematocrit of 24.3% (34.9–44.3%), MCV of 55.8 fL (81.8–96.9 fL), and red cell distribution width of 19.2%(12.3–15.6%). Peripheral blood smear showed anisocytosis, poikilocytosis, microcytosis, and hypochromia. Nutritional studies were as follows – Ferritin‐1.2 ng/mL (11–307 ng/mL), B12 level‐242 pg/mL (200–894 pg/mL), and serum folate‐10.2 ng/mL (3.1–17.5 ng/mL). Iron panel showed iron level of 10μg/mL (45–182μg/mL), total iron‐binding capacity (TIBC) of 441μg/dL (250–425μg/dL) and Transferrin saturation (Tsat) level of 2.3% (20–50%). She was given oral ferrous sulfate, which she was unable to tolerate and hence was started on weekly infusion of sodium ferric gluconate for 4 weeks. Her Hb responded appropriately over the course of 4 months. After 4 months of oral iron therapy, an EGD was performed. It did not show the narrowing of the proximal esophagus demonstrated previously on the EGD. To prevent excess blood loss during menses, gynecology team successfully bridged her with depot luprorelin until her menopause. On 2‐year follow‐up(in 2015), she continues to have Hb level stable around 13 gm/dL.

Esophagogram with Barium swallow images demonstrating proximal esophageal web (Initiation to emptying.

Koilonychia and Platynychia seen in multiple nails secondary to iron deficiency.

Koilonychia (spoon‐shaped nail) and platynychia (flat nails) are usually associated with chronic iron deficiency anemia and are more common in adults than children . The usual causes are iron deficiency secondary to gastrointestinal losses, nutritional deficiency secondary to poor intake, and/or malabsorption (e.g.,: celiac disease) or intestinal worms (e.g.,: Hookworm infestation) . Menorrhagia and menometrorrhagia are one of the commonest causes of chronic iron deficiency in women. Esophageal webs in patients with iron deficiency anemia are known as Plummer–Vinson syndrome or Patterson–Brown–Kelly syndrome . Though the pathogenesis of esophageal webs in iron deficiency remains unknown, it is postulated that the high cellular turnover rate in the epithelium of the upper digestive tract makes it vulnerable to iron deficiency because of the deficiency of the iron‐ dependent enzymes . Iron replacement therapy corrects the deficiency and leads to resolution of the webs. In conclusion, koilonychia or platynychia should prompt the clinician toward considering chronic iron deficiency as a cause of anemia.

Conflict of interest

None declared.