CLINICAL IMAGE
Spooning of the nails and webbing of the esophagus: koilonychia and Plummer–Vinson Syndrome Ankit Mangla1, Nikki Agarwal2, Jie Yu1 & Margaret Telfer3 1
Department of Internal Medicine, John H. Stroger Jr. Hospital of Cook County, 1901 West Harrison Street, Chicago, Illinois 60612 John H. Stroger Jr. Hospital of Cook County, 1926 West Harrison Street, Chicago, Illinois 60612 3 Department of Internal Medicine, Division of Hematology Oncology, John H. Stroger Jr. Hospital of Cook County, 1901 West Harrison Street, Chicago, Illinois 60612 2
Correspondence Ankit Mangla, Department of Internal Medicine, John H. Stroger Jr. Hospital of Cook County, 1901 West Harrison Street, Chicago, IL- 60612. Tel: +001 312 513 7437; Fax: +001 312 864 9537; E-mail: [email protected] Funding Information No sources of funding were declared for this study. Received: 14 May 2015; Revised: 2 August 2015; Accepted: 9 September 2015
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. Keywords Esophageal webs, iron deficiency anemia, koilonychia, Plummer Vinson Syndrome.
Clinical Case Reports 2015; 3(12): 1054– 1055 doi: 10.1002/ccr3.419 Consent: Written consent was obtained from the patient.
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. 1). 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. 2). Complete blood count showed white cell count of 4.6k/lL (4.4– 10.6k/lL), Hb of 6.8gm/dL (11.7–14.9gm/dL), hematocrit 1054
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 folate10.2 ng/mL (3.1–17.5 ng/mL). Iron panel showed iron level of 10lg/mL (45–182lg/mL), total iron-binding capacity (TIBC) of 441lg/dL (250–425lg/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
ª 2015 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd. This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.
A. Mangla et al.
Koilonychia and esophageal webs
Figure 1. Esophagogram with Barium swallow images demonstrating proximal esophageal web (Initiation to emptying.
Figure 2. Koilonychia and Platynychia seen in multiple nails secondary to iron deficiency.
follow-up(in 2015), she continues to have Hb level stable around 13 gm/dL. Koilonychia (spoon-shaped nail) and platynychia (flat nails) are usually associated with chronic iron deficiency anemia and are more common in adults than children [1]. 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) [2]. 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 [3]. 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 [3]. Iron replacement therapy corrects the deficiency and leads to resolution of the
ª 2015 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd.
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. References 1. Fawcett, R. S., S. Linford, and D. L. Stulberg. 2004. Nail abnormalities: clues to systemic disease. Am. Fam. Physician 69:1417–1424. 2. Kumar, G., L. Vaidyanathan, and L. G. Stead. 2007. Images in emergency medicine. Koilonychia, or spoon-shaped nails, is generally associated with iron-deficiency anemia. Ann. Emerg. Med. 49:243, 250. 3. Gude, D., D. P. Bansal, and A. Malu. 2013. Revisiting Plummer Vinson Syndrome. Ann. Med. Health Sci. Res. 3:119–121.
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Spooning of the nails and webbing of the esophagus: koilonychia and Plummer–Vinson Syndrome Ankit Mangla1, Nikki Agarwal2, Jie Yu1 & Margaret Telfer3 1
Department of Internal Medicine, John H. Stroger Jr. Hospital of Cook County, 1901 West Harrison Street, Chicago, Illinois 60612 John H. Stroger Jr. Hospital of Cook County, 1926 West Harrison Street, Chicago, Illinois 60612 3 Department of Internal Medicine, Division of Hematology Oncology, John H. Stroger Jr. Hospital of Cook County, 1901 West Harrison Street, Chicago, Illinois 60612 2
Correspondence Ankit Mangla, Department of Internal Medicine, John H. Stroger Jr. Hospital of Cook County, 1901 West Harrison Street, Chicago, IL- 60612. Tel: +001 312 513 7437; Fax: +001 312 864 9537; E-mail: [email protected] Funding Information No sources of funding were declared for this study. Received: 14 May 2015; Revised: 2 August 2015; Accepted: 9 September 2015
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. Keywords Esophageal webs, iron deficiency anemia, koilonychia, Plummer Vinson Syndrome.
Clinical Case Reports 2015; 3(12): 1054– 1055 doi: 10.1002/ccr3.419 Consent: Written consent was obtained from the patient.
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. 1). 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. 2). Complete blood count showed white cell count of 4.6k/lL (4.4– 10.6k/lL), Hb of 6.8gm/dL (11.7–14.9gm/dL), hematocrit 1054
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 folate10.2 ng/mL (3.1–17.5 ng/mL). Iron panel showed iron level of 10lg/mL (45–182lg/mL), total iron-binding capacity (TIBC) of 441lg/dL (250–425lg/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
ª 2015 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd. This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.
A. Mangla et al.
Koilonychia and esophageal webs
Figure 1. Esophagogram with Barium swallow images demonstrating proximal esophageal web (Initiation to emptying.
Figure 2. Koilonychia and Platynychia seen in multiple nails secondary to iron deficiency.
follow-up(in 2015), she continues to have Hb level stable around 13 gm/dL. Koilonychia (spoon-shaped nail) and platynychia (flat nails) are usually associated with chronic iron deficiency anemia and are more common in adults than children [1]. 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) [2]. 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 [3]. 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 [3]. Iron replacement therapy corrects the deficiency and leads to resolution of the
ª 2015 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd.
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. References 1. Fawcett, R. S., S. Linford, and D. L. Stulberg. 2004. Nail abnormalities: clues to systemic disease. Am. Fam. Physician 69:1417–1424. 2. Kumar, G., L. Vaidyanathan, and L. G. Stead. 2007. Images in emergency medicine. Koilonychia, or spoon-shaped nails, is generally associated with iron-deficiency anemia. Ann. Emerg. Med. 49:243, 250. 3. Gude, D., D. P. Bansal, and A. Malu. 2013. Revisiting Plummer Vinson Syndrome. Ann. Med. Health Sci. Res. 3:119–121.
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